Creatine kinase mitochondrial form. Medical search

A transferase that catalyzes formation of PHOSPHOCREATINE from ATP + CREATINE. The reaction stores ATP energy as phosphocreatine. Three cytoplasmic ISOENZYMES have been identified in human tissues: the MM type from SKELETAL MUSCLE, the MB type from myocardial tissue and the BB type from nervous tissue as well as a mitochondrial isoenzyme. Macro-creatine kinase refers to creatine kinase complexed with other serum proteins.

A form of creatine kinase found in the MITOCHONDRIA.

An amino acid that occurs in vertebrate tissues and in urine. In muscle tissue, creatine generally occurs as phosphocreatine. Creatine is excreted as CREATININE in the urine.

Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)

Structurally related forms of an enzyme. Each isoenzyme has the same mechanism and classification, but differs in its chemical, physical, or immunological characteristics.

An isoenzyme of creatine kinase found in the MUSCLE.

Intracellular fluid from the cytoplasm after removal of ORGANELLES and other insoluble cytoplasmic components.

Mitochondria in hepatocytes. As in all mitochondria, there are an outer membrane and an inner membrane, together creating two separate mitochondrial compartments: the internal matrix space and a much narrower intermembrane space. In the liver mitochondrion, an estimated 67% of the total mitochondrial proteins is located in the matrix. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p343-4)

A form of creatine kinase found in the BRAIN.

Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.

The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.

The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.

An isoenzyme of creatine kinase found in the CARDIAC MUSCLE.

A species of the genus SACCHAROMYCES, family Saccharomycetaceae, order Saccharomycetales, known as "baker's" or "brewer's" yeast. The dried form is used as a dietary supplement.

Phosphotransferases that catalyzes the conversion of 1-phosphatidylinositol to 1-phosphatidylinositol 3-phosphate. Many members of this enzyme class are involved in RECEPTOR MEDIATED SIGNAL TRANSDUCTION and regulation of vesicular transport with the cell. Phosphatidylinositol 3-Kinases have been classified both according to their substrate specificity and their mode of action within the cell.

Analyses for a specific enzyme activity, or of the level of a specific enzyme that is used to assess health and disease risk, for early detection of disease or disease prediction, diagnosis, and change in disease status.

An endogenous substance found mainly in skeletal muscle of vertebrates. It has been tried in the treatment of cardiac disorders and has been added to cardioplegic solutions. (Reynolds JEF(Ed): Martindale: The Extra Pharmacopoeia (electronic version). Micromedex, Inc, Englewood, CO, 1996)

An intracellular signaling system involving the MAP kinase cascades (three-membered protein kinase cascades). Various upstream activators, which act in response to extracellular stimuli, trigger the cascades by activating the first member of a cascade, MAP KINASE KINASE KINASES; (MAPKKKs). Activated MAPKKKs phosphorylate MITOGEN-ACTIVATED PROTEIN KINASE KINASES which in turn phosphorylate the MITOGEN-ACTIVATED PROTEIN KINASES; (MAPKs). The MAPKs then act on various downstream targets to affect gene expression. In mammals, there are several distinct MAP kinase pathways including the ERK (extracellular signal-regulated kinase) pathway, the SAPK/JNK (stress-activated protein kinase/c-jun kinase) pathway, and the p38 kinase pathway. There is some sharing of components among the pathways depending on which stimulus originates activation of the cascade.

A family of enzymes that catalyze the conversion of ATP and a protein to ADP and a phosphoprotein.

A group of enzymes that catalyzes the phosphorylation of serine or threonine residues in proteins, with ATP or other nucleotides as phosphate donors.

An enzyme that catalyzes the phosphorylation of the guanidine nitrogen of arginine in the presence of ATP and a divalent cation with formation of phosphorylarginine and ADP. EC 2.7.3.3.

Contractile tissue that produces movement in animals.

Agents that inhibit PROTEIN KINASES.

A CALMODULIN-dependent enzyme that catalyzes the phosphorylation of proteins. This enzyme is also sometimes dependent on CALCIUM. A wide range of proteins can act as acceptor, including VIMENTIN; SYNAPSINS; GLYCOGEN SYNTHASE; MYOSIN LIGHT CHAINS; and the MICROTUBULE-ASSOCIATED PROTEINS. (From Enzyme Nomenclature, 1992, p277)

A PROTEIN-TYROSINE KINASE family that was originally identified by homology to the Rous sarcoma virus ONCOGENE PROTEIN PP60(V-SRC). They interact with a variety of cell-surface receptors and participate in intracellular signal transduction pathways. Oncogenic forms of src-family kinases can occur through altered regulation or expression of the endogenous protein and by virally encoded src (v-src) genes.

A tetrameric enzyme that, along with the coenzyme NAD+, catalyzes the interconversion of LACTATE and PYRUVATE. In vertebrates, genes for three different subunits (LDH-A, LDH-B and LDH-C) exist.

The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.

An serine-threonine protein kinase that requires the presence of physiological concentrations of CALCIUM and membrane PHOSPHOLIPIDS. The additional presence of DIACYLGLYCEROLS markedly increases its sensitivity to both calcium and phospholipids. The sensitivity of the enzyme can also be increased by PHORBOL ESTERS and it is believed that protein kinase C is the receptor protein of tumor-promoting phorbol esters.

Necrosis or disintegration of skeletal muscle often followed by myoglobinuria.

A mitogen-activated protein kinase subfamily that regulates a variety of cellular processes including CELL GROWTH PROCESSES; CELL DIFFERENTIATION; APOPTOSIS; and cellular responses to INFLAMMATION. The P38 MAP kinases are regulated by CYTOKINE RECEPTORS and can be activated in response to bacterial pathogens.

A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.

Electrophoresis in which cellulose acetate is the diffusion medium.

An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.

A group of enzymes that are dependent on CYCLIC AMP and catalyze the phosphorylation of SERINE or THREONINE residues on proteins. Included under this category are two cyclic-AMP-dependent protein kinase subtypes, each of which is defined by its subunit composition.

The rate dynamics in chemical or physical systems.

A proline-directed serine/threonine protein kinase which mediates signal transduction from the cell surface to the nucleus. Activation of the enzyme by phosphorylation leads to its translocation into the nucleus where it acts upon specific transcription factors. p40 MAPK and p41 MAPK are isoforms.

ATP:pyruvate 2-O-phosphotransferase. A phosphotransferase that catalyzes reversibly the phosphorylation of pyruvate to phosphoenolpyruvate in the presence of ATP. It has four isozymes (L, R, M1, and M2). Deficiency of the enzyme results in hemolytic anemia. EC 2.7.1.40.

An enzyme that catalyzes the phosphorylation of AMP to ADP in the presence of ATP or inorganic triphosphate. EC 2.7.4.3.

A family of serine-threonine kinases that bind to and are activated by MONOMERIC GTP-BINDING PROTEINS such as RAC GTP-BINDING PROTEINS and CDC42 GTP-BINDING PROTEIN. They are intracellular signaling kinases that play a role the regulation of cytoskeletal organization.

Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.

A serine-threonine protein kinase family whose members are components in protein kinase cascades activated by diverse stimuli. These MAPK kinases phosphorylate MITOGEN-ACTIVATED PROTEIN KINASES and are themselves phosphorylated by MAP KINASE KINASE KINASES. JNK kinases (also known as SAPK kinases) are a subfamily.

A subgroup of mitogen-activated protein kinases that activate TRANSCRIPTION FACTOR AP-1 via the phosphorylation of C-JUN PROTEINS. They are components of intracellular signaling pathways that regulate CELL PROLIFERATION; APOPTOSIS; and CELL DIFFERENTIATION.

This enzyme catalyzes the last step of CREATINE biosynthesis by catalyzing the METHYLATION of guanidinoacetate to CREATINE.

A 44-kDa extracellular signal-regulated MAP kinase that may play a role the initiation and regulation of MEIOSIS; MITOSIS; and postmitotic functions in differentiated cells. It phosphorylates a number of TRANSCRIPTION FACTORS; and MICROTUBULE-ASSOCIATED PROTEINS.

Acquired, familial, and congenital disorders of SKELETAL MUSCLE and SMOOTH MUSCLE.

Spectroscopic method of measuring the magnetic moment of elementary particles such as atomic nuclei, protons or electrons. It is employed in clinical applications such as NMR Tomography (MAGNETIC RESONANCE IMAGING).

Enzymes of a subclass of TRANSFERASES that catalyze the transfer of an amidino group from donor to acceptor. EC 2.1.4.

Protein kinases that catalyze the PHOSPHORYLATION of TYROSINE residues in proteins with ATP or other nucleotides as phosphate donors.

The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.

A conjugated protein which is the oxygen-transporting pigment of muscle. It is made up of one globin polypeptide chain and one heme group.

Elements of limited time intervals, contributing to particular results or situations.

Phosphoprotein with protein kinase activity that functions in the G2/M phase transition of the CELL CYCLE. It is the catalytic subunit of the MATURATION-PROMOTING FACTOR and complexes with both CYCLIN A and CYCLIN B in mammalian cells. The maximal activity of cyclin-dependent kinase 1 is achieved when it is fully dephosphorylated.

Protein kinases that control cell cycle progression in all eukaryotes and require physical association with CYCLINS to achieve full enzymatic activity. Cyclin-dependent kinases are regulated by phosphorylation and dephosphorylation events.

The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety.

Mitogen-activated protein kinase kinase kinases (MAPKKKs) are serine-threonine protein kinases that initiate protein kinase signaling cascades. They phosphorylate MITOGEN-ACTIVATED PROTEIN KINASE KINASES; (MAPKKs) which in turn phosphorylate MITOGEN-ACTIVATED PROTEIN KINASES; (MAPKs).

NECROSIS of the MYOCARDIUM caused by an obstruction of the blood supply to the heart (CORONARY CIRCULATION).

A dsRNA-activated cAMP-independent protein serine/threonine kinase that is induced by interferon. In the presence of dsRNA and ATP, the kinase autophosphorylates on several serine and threonine residues. The phosphorylated enzyme catalyzes the phosphorylation of the alpha subunit of EUKARYOTIC INITIATION FACTOR-2, leading to the inhibition of protein synthesis.

A ubiquitous casein kinase that is comprised of two distinct catalytic subunits and dimeric regulatory subunit. Casein kinase II has been shown to phosphorylate a large number of substrates, many of which are proteins involved in the regulation of gene expression.

A group of protein-serine-threonine kinases that was originally identified as being responsible for the PHOSPHORYLATION of CASEINS. They are ubiquitous enzymes that have a preference for acidic proteins. Casein kinases play a role in SIGNAL TRANSDUCTION by phosphorylating a variety of regulatory cytoplasmic and regulatory nuclear proteins.

The mitochondria of the myocardium.

A family of protein serine/threonine kinases which act as intracellular signalling intermediates. Ribosomal protein S6 kinases are activated through phosphorylation in response to a variety of HORMONES and INTERCELLULAR SIGNALING PEPTIDES AND PROTEINS. Phosphorylation of RIBOSOMAL PROTEIN S6 by enzymes in this class results in increased expression of 5' top MRNAs. Although specific for RIBOSOMAL PROTEIN S6 members of this class of kinases can act on a number of substrates within the cell. The immunosuppressant SIROLIMUS inhibits the activation of ribosomal protein S6 kinases.

An abundant 43-kDa mitogen-activated protein kinase kinase subtype with specificity for MITOGEN-ACTIVATED PROTEIN KINASE 1 and MITOGEN-ACTIVATED PROTEIN KINASE 3.

Inflammation of a muscle or muscle tissue.

The chemical reactions involved in the production and utilization of various forms of energy in cells.

Enzymes of the transferase class that catalyze the conversion of L-aspartate and 2-ketoglutarate to oxaloacetate and L-glutamate. EC 2.6.1.1.

A class of cellular receptors that have an intrinsic PROTEIN-TYROSINE KINASE activity.

An enzyme that catalyzes the conversion of ATP and thymidine to ADP and thymidine 5'-phosphate. Deoxyuridine can also act as an acceptor and dGTP as a donor. (From Enzyme Nomenclature, 1992) EC 2.7.1.21.

Electrophoresis in which agar or agarose gel is used as the diffusion medium.

The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.

A mitogen-activated protein kinase subfamily that is widely expressed and plays a role in regulation of MEIOSIS; MITOSIS; and post mitotic functions in differentiated cells. The extracellular signal regulated MAP kinases are regulated by a broad variety of CELL SURFACE RECEPTORS and can be activated by certain CARCINOGENS.

A mitogen-activated protein kinase kinase with specificity for JNK MITOGEN-ACTIVATED PROTEIN KINASES; P38 MITOGEN-ACTIVATED PROTEIN KINASES and the RETINOID X RECEPTORS. It takes part in a SIGNAL TRANSDUCTION pathway that is activated in response to cellular stress.

The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.

One of the three polypeptide chains that make up the TROPONIN complex. It is a cardiac-specific protein that binds to TROPOMYOSIN. It is released from damaged or injured heart muscle cells (MYOCYTES, CARDIAC). Defects in the gene encoding troponin T result in FAMILIAL HYPERTROPHIC CARDIOMYOPATHY.

One of the three polypeptide chains that make up the TROPONIN complex. It inhibits F-actin-myosin interactions.

The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.

An enzyme that catalyzes the conversion of phosphatidylinositol (PHOSPHATIDYLINOSITOLS) to phosphatidylinositol 4-phosphate, the first committed step in the biosynthesis of phosphatidylinositol 4,5-bisphosphate.

A group of enzymes that transfers a phosphate group onto an alcohol group acceptor. EC 2.7.1.

A superfamily of PROTEIN-SERINE-THREONINE KINASES that are activated by diverse stimuli via protein kinase cascades. They are the final components of the cascades, activated by phosphorylation by MITOGEN-ACTIVATED PROTEIN KINASE KINASES, which in turn are activated by mitogen-activated protein kinase kinase kinases (MAP KINASE KINASE KINASES).

A family of cell cycle-dependent kinases that are related in structure to CDC28 PROTEIN KINASE; S CEREVISIAE; and the CDC2 PROTEIN KINASE found in mammalian species.

Conversion of an inactive form of an enzyme to one possessing metabolic activity. It includes 1, activation by ions (activators); 2, activation by cofactors (coenzymes); and 3, conversion of an enzyme precursor (proenzyme or zymogen) to an active enzyme.

A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS.

Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.

A protein serine-threonine kinase that catalyzes the PHOSPHORYLATION of I KAPPA B PROTEINS. This enzyme also activates the transcription factor NF-KAPPA B and is composed of alpha and beta catalytic subunits, which are protein kinases and gamma, a regulatory subunit.

A glycogen synthase kinase that was originally described as a key enzyme involved in glycogen metabolism. It regulates a diverse array of functions such as CELL DIVISION, microtubule function and APOPTOSIS.

A family of highly conserved serine-threonine kinases that are involved in the regulation of MITOSIS. They are involved in many aspects of cell division, including centrosome duplication, SPINDLE APPARATUS formation, chromosome alignment, attachment to the spindle, checkpoint activation, and CYTOKINESIS.

A group of intracellular-signaling serine threonine kinases that bind to RHO GTP-BINDING PROTEINS. They were originally found to mediate the effects of rhoA GTP-BINDING PROTEIN on the formation of STRESS FIBERS and FOCAL ADHESIONS. Rho-associated kinases have specificity for a variety of substrates including MYOSIN-LIGHT-CHAIN PHOSPHATASE and LIM KINASES.

The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.

Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.

The parts of a macromolecule that directly participate in its specific combination with another molecule.

A ubiquitously expressed protein kinase that is involved in a variety of cellular SIGNAL PATHWAYS. Its activity is regulated by a variety of signaling protein tyrosine kinase.

A cytoplasmic serine threonine kinase involved in regulating CELL DIFFERENTIATION and CELLULAR PROLIFERATION. Overexpression of this enzyme has been shown to promote PHOSPHORYLATION of BCL-2 PROTO-ONCOGENE PROTEINS and chemoresistance in human acute leukemia cells.

Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.

Established cell cultures that have the potential to propagate indefinitely.

Intracellular signaling protein kinases that play a signaling role in the regulation of cellular energy metabolism. Their activity largely depends upon the concentration of cellular AMP which is increased under conditions of low energy or metabolic stress. AMP-activated protein kinases modify enzymes involved in LIPID METABOLISM, which in turn provide substrates needed to convert AMP into ATP.

A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.

Products of proto-oncogenes. Normally they do not have oncogenic or transforming properties, but are involved in the regulation or differentiation of cell growth. They often have protein kinase activity.

Damage to the MYOCARDIUM resulting from MYOCARDIAL REPERFUSION (restoration of blood flow to ischemic areas of the HEART.) Reperfusion takes place when there is spontaneous thrombolysis, THROMBOLYTIC THERAPY, collateral flow from other coronary vascular beds, or reversal of vasospasm.

The hollow, muscular organ that maintains the circulation of the blood.

A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.

RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.

An enzyme of the transferase class that uses ATP to catalyze the phosphorylation of diacylglycerol to a phosphatidate. EC 2.7.1.107.

The protein constituents of muscle, the major ones being ACTINS and MYOSINS. More than a dozen accessory proteins exist including TROPONIN; TROPOMYOSIN; and DYSTROPHIN.

Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.

The long cylindrical contractile organelles of STRIATED MUSCLE cells composed of ACTIN FILAMENTS; MYOSIN filaments; and other proteins organized in arrays of repeating units called SARCOMERES .

A non-essential amino acid. In animals it is synthesized from PHENYLALANINE. It is also the precursor of EPINEPHRINE; THYROID HORMONES; and melanin.

A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.

A protein-serine-threonine kinase that is activated by PHOSPHORYLATION in response to GROWTH FACTORS or INSULIN. It plays a major role in cell metabolism, growth, and survival as a core component of SIGNAL TRANSDUCTION. Three isoforms have been described in mammalian cells.

A non-receptor protein tyrosine kinase that is localized to FOCAL ADHESIONS and is a central component of integrin-mediated SIGNAL TRANSDUCTION PATHWAYS. Focal adhesion kinase 1 interacts with PAXILLIN and undergoes PHOSPHORYLATION in response to adhesion of cell surface integrins to the EXTRACELLULAR MATRIX. Phosphorylated p125FAK protein binds to a variety of SH2 DOMAIN and SH3 DOMAIN containing proteins and helps regulate CELL ADHESION and CELL MIGRATION.

A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.

Proteins prepared by recombinant DNA technology.

Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.

An enzyme that phosphorylates myosin light chains in the presence of ATP to yield myosin-light chain phosphate and ADP, and requires calcium and CALMODULIN. The 20-kDa light chain is phosphorylated more rapidly than any other acceptor, but light chains from other myosins and myosin itself can act as acceptors. The enzyme plays a central role in the regulation of smooth muscle contraction.

Generally, restoration of blood supply to heart tissue which is ischemic due to decrease in normal blood supply. The decrease may result from any source including atherosclerotic obstruction, narrowing of the artery, or surgical clamping. Reperfusion can be induced to treat ischemia. Methods include chemical dissolution of an occluding thrombus, administration of vasodilator drugs, angioplasty, catheterization, and artery bypass graft surgery. However, it is thought that reperfusion can itself further damage the ischemic tissue, causing MYOCARDIAL REPERFUSION INJURY.

Common name for the species Gallus gallus, the domestic fowl, in the family Phasianidae, order GALLIFORMES. It is descended from the red jungle fowl of SOUTHEAST ASIA.

Compounds which restore enzymatic activity by removing an inhibitory group bound to the reactive site of the enzyme.

A Janus kinase subtype that is involved in signaling from GROWTH HORMONE RECEPTORS; PROLACTIN RECEPTORS; and a variety of CYTOKINE RECEPTORS such as ERYTHROPOIETIN RECEPTORS and INTERLEUKIN RECEPTORS. Dysregulation of Janus kinase 2 due to GENETIC TRANSLOCATIONS have been associated with a variety of MYELOPROLIFERATIVE DISORDERS.

The relationship between the dose of an administered drug and the response of the organism to the drug.

A family of non-receptor, PROLINE-rich protein-tyrosine kinases.

Theoretical representations that simulate the behavior or activity of biological processes or diseases. For disease models in living animals, DISEASE MODELS, ANIMAL is available. Biological models include the use of mathematical equations, computers, and other electronic equipment.

Recombinant proteins produced by the GENETIC TRANSLATION of fused genes formed by the combination of NUCLEIC ACID REGULATORY SEQUENCES of one or more genes with the protein coding sequences of one or more genes.

A family of ribosomal protein S6 kinases that are structurally distinguished from RIBOSOMAL PROTEIN S6 KINASES, 70-KDA by their apparent molecular size and the fact they contain two functional kinase domains. Although considered RIBOSOMAL PROTEIN S6 KINASES, members of this family are activated via the MAP KINASE SIGNALING SYSTEM and have been shown to act on a diverse array of substrates that are involved in cellular regulation such as RIBOSOMAL PROTEIN S6 and CAMP RESPONSE ELEMENT-BINDING PROTEIN.

One of the mechanisms by which CELL DEATH occurs (compare with NECROSIS and AUTOPHAGOCYTOSIS). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA; (DNA FRAGMENTATION); at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth.

Proteins and peptides that are involved in SIGNAL TRANSDUCTION within the cell. Included here are peptides and proteins that regulate the activity of TRANSCRIPTION FACTORS and cellular processes in response to signals from CELL SURFACE RECEPTORS. Intracellular signaling peptide and proteins may be part of an enzymatic signaling cascade or act through binding to and modifying the action of other signaling factors.

A protein kinase C subtype that was originally characterized as a CALCIUM-independent, serine-threonine kinase that is activated by PHORBOL ESTERS and DIACYLGLYCEROLS. It is targeted to specific cellular compartments in response to extracellular signals that activate G-PROTEIN-COUPLED RECEPTORS; TYROSINE KINASE RECEPTORS; and intracellular protein tyrosine kinase.

The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.

Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control of gene action in enzyme synthesis.

One of the minor protein components of skeletal muscle. Its function is to serve as the calcium-binding component in the troponin-tropomyosin B-actin-myosin complex by conferring calcium sensitivity to the cross-linked actin and myosin filaments.

A 195-kDa MAP kinase kinase kinase with broad specificity for MAP KINASE KINASES. It is found localized in the CYTOSKELETON and can activate a variety of MAP kinase-dependent pathways.

Method of analyzing chemicals using automation.

Mitochondria of skeletal and smooth muscle. It does not include myocardial mitochondria for which MITOCHONDRIA, HEART is available.

A multifunctional calcium-calmodulin-dependent protein kinase subtype that occurs as an oligomeric protein comprised of twelve subunits. It differs from other enzyme subtypes in that it lacks a phosphorylatable activation domain that can respond to CALCIUM-CALMODULIN-DEPENDENT PROTEIN KINASE KINASE.

The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.

Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.

An alkylating sulfhydryl reagent. Its actions are similar to those of iodoacetate.

PKC beta encodes two proteins (PKCB1 and PKCBII) generated by alternative splicing of C-terminal exons. It is widely distributed with wide-ranging roles in processes such as B-cell receptor regulation, oxidative stress-induced apoptosis, androgen receptor-dependent transcriptional regulation, insulin signaling, and endothelial cell proliferation.

Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.

The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.

Recording of the moment-to-moment electromotive forces of the HEART as projected onto various sites on the body's surface, delineated as a scalar function of time. The recording is monitored by a tracing on slow moving chart paper or by observing it on a cardioscope, which is a CATHODE RAY TUBE DISPLAY.

An enzyme catalyzing the transfer of a phosphate group from 3-phospho-D-glycerate in the presence of ATP to yield 3-phospho-D-glyceroyl phosphate and ADP. EC 2.7.2.3.

A phorbol ester found in CROTON OIL with very effective tumor promoting activity. It stimulates the synthesis of both DNA and RNA.

The range or frequency distribution of a measurement in a population (of organisms, organs or things) that has not been selected for the presence of disease or abnormality.

A 44 kDa mitogen-activated protein kinase kinase with specificity for MITOGEN-ACTIVATED PROTEIN KINASE 1 and MITOGEN-ACTIVATED PROTEIN KINASE 3.

A disorder of cardiac function caused by insufficient blood flow to the muscle tissue of the heart. The decreased blood flow may be due to narrowing of the coronary arteries (CORONARY ARTERY DISEASE), to obstruction by a thrombus (CORONARY THROMBOSIS), or less commonly, to diffuse narrowing of arterioles and other small vessels within the heart. Severe interruption of the blood supply to the myocardial tissue may result in necrosis of cardiac muscle (MYOCARDIAL INFARCTION).

A serine threonine kinase that controls a wide range of growth-related cellular processes. The protein is referred to as the target of RAPAMYCIN due to the discovery that SIROLIMUS (commonly known as rapamycin) forms an inhibitory complex with TACROLIMUS BINDING PROTEIN 1A that blocks the action of its enzymatic activity.

A key regulator of CELL CYCLE progression. It partners with CYCLIN E to regulate entry into S PHASE and also interacts with CYCLIN A to phosphorylate RETINOBLASTOMA PROTEIN. Its activity is inhibited by CYCLIN-DEPENDENT KINASE INHIBITOR P27 and CYCLIN-DEPENDENT KINASE INHIBITOR P21.

A non-essential amino acid occurring in natural form as the L-isomer. It is synthesized from GLYCINE or THREONINE. It is involved in the biosynthesis of PURINES; PYRIMIDINES; and other amino acids.

A group of cyclic GMP-dependent enzymes that catalyze the phosphorylation of SERINE or THREONINE residues of proteins.

Derivatives of the steroid androstane having two double bonds at any site in any of the rings.

Inorganic salts of phosphoric acid.

A family of iminourea derivatives. The parent compound has been isolated from mushrooms, corn germ, rice hulls, mussels, earthworms, and turnip juice. Derivatives may have antiviral and antifungal properties.

A metallic element that has the atomic symbol Mg, atomic number 12, and atomic weight 24.31. It is important for the activity of many enzymes, especially those involved in OXIDATIVE PHOSPHORYLATION.

An enzyme that catalyzes the conversion of ATP and PHOSPHORYLASE B to ADP and PHOSPHORYLASE A.

Immunologic method used for detecting or quantifying immunoreactive substances. The substance is identified by first immobilizing it by blotting onto a membrane and then tagging it with labeled antibodies.

A serine-threonine kinase that plays important roles in CELL DIFFERENTIATION; CELL MIGRATION; and CELL DEATH of NERVE CELLS. It is closely related to other CYCLIN-DEPENDENT KINASES but does not seem to participate in CELL CYCLE regulation.

Iodinated derivatives of acetic acid. Iodoacetates are commonly used as alkylating sulfhydryl reagents and enzyme inhibitors in biochemical research.

Cells grown in vitro from neoplastic tissue. If they can be established as a TUMOR CELL LINE, they can be propagated in cell culture indefinitely.

A cell line derived from cultured tumor cells.

The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)

Electron transfer through the cytochrome system liberating free energy which is transformed into high-energy phosphate bonds.

Technique by which phase transitions of chemical reactions can be followed by observation of the heat absorbed or liberated.

The sum of the weight of all the atoms in a molecule.

The fission of a CELL. It includes CYTOKINESIS, when the CYTOPLASM of a cell is divided, and CELL NUCLEUS DIVISION.

Progressive restriction of the developmental potential and increasing specialization of function that leads to the formation of specialized cells, tissues, and organs.

An electrochemical process in which macromolecules or colloidal particles with a net electric charge migrate in a solution under the influence of an electric current.

Separation technique in which the stationary phase consists of ion exchange resins. The resins contain loosely held small ions that easily exchange places with other small ions of like charge present in solutions washed over the resins.

An activity in which the body is propelled by moving the legs rapidly. Running is performed at a moderate to rapid pace and should be differentiated from JOGGING, which is performed at a much slower pace.

A method of chemical analysis based on the detection of characteristic radionuclides following a nuclear bombardment. It is also known as radioactivity analysis. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)

Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.

Serologic tests in which a positive reaction manifested by visible CHEMICAL PRECIPITATION occurs when a soluble ANTIGEN reacts with its precipitins, i.e., ANTIBODIES that can form a precipitate.

Proteins that control the CELL DIVISION CYCLE. This family of proteins includes a wide variety of classes, including CYCLIN-DEPENDENT KINASES, mitogen-activated kinases, CYCLINS, and PHOSPHOPROTEIN PHOSPHATASES as well as their putative substrates such as chromatin-associated proteins, CYTOSKELETAL PROTEINS, and TRANSCRIPTION FACTORS.

An enzyme that catalyzes reversible reactions of a nucleoside triphosphate, e.g., ATP, with a nucleoside monophosphate, e.g., UMP, to form ADP and UDP. Many nucleoside monophosphates can act as acceptor while many ribo- and deoxyribonucleoside triphosphates can act as donor. EC 2.7.4.4.

Compounds and molecular complexes that consist of very large numbers of atoms and are generally over 500 kDa in size. In biological systems macromolecular substances usually can be visualized using ELECTRON MICROSCOPY and are distinguished from ORGANELLES by the lack of a membrane structure.

A mitogen-activated protein kinase kinase with specificity for P38 MITOGEN-ACTIVATED PROTEIN KINASES.

The rate at which oxygen is used by a tissue; microliters of oxygen STPD used per milligram of tissue per hour; the rate at which oxygen enters the blood from alveolar gas, equal in the steady state to the consumption of oxygen by tissue metabolism throughout the body. (Stedman, 25th ed, p346)

Proteins which bind to DNA. The family includes proteins which bind to both double- and single-stranded DNA and also includes specific DNA binding proteins in serum which can be used as markers for malignant diseases.

An examination of chemicals in the blood.

An adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH.

A compound tubular gland, located around the eyes and nasal passages in marine animals and birds, the physiology of which figures in water-electrolyte balance. The Pekin duck serves as a common research animal in salt gland studies. A rectal gland or rectal salt gland in the dogfish shark is attached at the junction of the intestine and cloaca and aids the kidneys in removing excess salts from the blood. (Storer, Usinger, Stebbins & Nybakken: General Zoology, 6th ed, p658)

A casein kinase that was originally described as a monomeric enzyme with a molecular weight of 30-40 kDa. Several ISOENZYMES of casein kinase I have been found which are encoded by separate genes. Many of the casein kinase I isoenzymes have been shown to play distinctive roles in intracellular SIGNAL TRANSDUCTION.

Irritants and reagents for labeling terminal amino acid groups.

A mitogen-activated protein kinase kinase with specificity for a subset of P38 MITOGEN-ACTIVATED PROTEIN KINASES that includes MITOGEN-ACTIVATED PROTEIN KINASE 12; MITOGEN-ACTIVATED PROTEIN KINASE 13; and MITOGEN-ACTIVATED PROTEIN KINASE 14.

A c-jun amino-terminal kinase that is activated by environmental stress and pro-inflammatory cytokines. Several isoforms of the protein with molecular sizes of 43 and 48 KD exist due to multiple ALTERNATIVE SPLICING.

A technique using antibodies for identifying or quantifying a substance. Usually the substance being studied serves as antigen both in antibody production and in measurement of antibody by the test substance.

Large, multinucleate single cells, either cylindrical or prismatic in shape, that form the basic unit of SKELETAL MUSCLE. They consist of MYOFIBRILS enclosed within and attached to the SARCOLEMMA. They are derived from the fusion of skeletal myoblasts (MYOBLASTS, SKELETAL) into a syncytium, followed by differentiation.

An aurora kinase that localizes to the CENTROSOME during MITOSIS and is involved in centrosome regulation and formation of the MITOTIC SPINDLE. Aurora A overexpression in many malignant tumor types suggests that it may be directly involved in NEOPLASTIC CELL TRANSFORMATION.

Highly conserved protein-serine threonine kinases that phosphorylate and activate a group of AGC protein kinases, especially in response to the production of the SECOND MESSENGERS, phosphatidylinositol 3,4,-biphosphate (PtdIns(3,4)P2) and phosphatidylinositol 3,4,5-triphosphate (PtdIns(3,4,5)P3).

A non-receptor protein-tyrosine kinase that is expressed primarily in the BRAIN; OSTEOBLASTS; and LYMPHOID CELLS. In the CENTRAL NERVOUS SYSTEM focal adhesion kinase 2 modulates ION CHANNEL function and MITOGEN-ACTIVATED PROTEIN KINASES activity.

The biosynthesis of RNA carried out on a template of DNA. The biosynthesis of DNA from an RNA template is called REVERSE TRANSCRIPTION.

Heart failure affects mitochondrial but not myofibrillar intrinsic properties of skeletal muscle. (1/49)

BACKGROUND: Congestive heart failure (CHF) induces abnormalities in skeletal muscle that are thought to in part explain exercise intolerance. The aim of the present study was to determine whether these changes actually result in contractile or metabolic functional alterations and whether they are muscle type specific. METHODS AND RESULTS: With a rat model of CHF (induced by aortic banding), we studied mitochondrial function, mechanical properties, and creatine kinase (CK) compartmentation in situ in permeabilized fibers from soleus (SOL), an oxidative slow-twitch muscle, and white gastrocnemius (GAS), a glycolytic fast-twitch muscle. Animals were studied 7 months after surgery, and CHF was documented on the basis of anatomic data. Alterations in skeletal muscle phenotype were documented with an increased proportion of fast-type fiber and fast myosin heavy chain, decreased capillary-to-fiber ratio, and decreased citrate synthase activity. Despite a slow-to-fast phenotype transition in SOL, no change was observed in contractile capacity or calcium sensitivity. However, muscles from CHF rats exhibited a dramatic decrease in oxidative capacities (oxygen consumption per gram of fiber dry weight) of 35% for SOL and 45% for GAS (P:<0.001). Moreover, the regulation of respiration with ADP and mitochondrial CK and adenylate kinase was impaired in CHF SOL. Mitochondrial CK activity and content (Western blots) were dramatically decreased in both muscles. CONCLUSIONS: CHF results in alterations in both mitochondrial function and phosphotransfer systems but unchanged myofibrillar function in skeletal muscles, which suggests a myopathy of metabolic origin in CHF. (+info)

Changes in mRNA expression profile underlie phenotypic adaptations in creatine kinase-deficient muscles. (2/49)

We have studied the mechanisms that regulate the remodeling of the glycolytic, mitochondrial and structural network of muscles of creatine kinase M (M-CK)/sarcomeric mitochondrial creatine kinase (ScCKmit) knockout mice by comparison of wild-type and mutant mRNA profiles on cDNA arrays. The magnitudes of changes in mRNA levels were most prominent in M-CK/ScCKmit (CK(-/-)) double mutants but did never exceed those of previously observed changes in protein level for any protein examined. In gastrocnemius of CK(-/-) mice we measured a 2.5-fold increase in mRNA level for mitochondrial encoded cytochrome c oxidase (COX)-III which corresponds to the increase in protein content. The level of the nuclear encoded mRNAs for COX-IV, H(+)-ATP synthase-C, adenine nucleotide translocator-1 and insulin-regulatable glucose transporter-4 showed a 1.5-fold increase, also in agreement with protein data. In contrast, no concomitant up-regulation in mRNA and protein content was detected for the mitochondrial inorganic phosphate-carrier, voltage-dependent anion channel and certain glycolytic enzymes. Our results reveal that regulation of transcript level plays an important role, but it is not the only principle involved in the remodeling of mitochondrial and cytosolic design of CK(-/-) muscles. (+info)

Mitochondrial creatine kinase: properties and function. (3/49)

This review describes properties of mitochondrial creatine kinase from heart and skeletal muscle studied in the author's group at the Department of Biochemistry of Moscow State University. The results are compared to the data in the literature. The author's point of view on the physiological role of mitochondrial creatine kinase is presented. (+info)

Multiple interference of anthracyclines with mitochondrial creatine kinases: preferential damage of the cardiac isoenzyme and its implications for drug cardiotoxicity. (4/49)

Anthracyclines are among the most efficient drugs of cancer chemotherapy, but their use is limited by a significant risk of cardiotoxicity, which is still far from being understood. This study investigates whether impairment of mitochondrial creatine kinase (MtCK), a key enzyme in cellular energy metabolism, could be involved in anthracycline cardiotoxicity. We have analyzed the effects of three anthracyclines, doxorubicin, daunorubicin, and idarubicin, on two MtCK isoenzymes, sarcomeric/cardiac sMtCK and ubiquitous uMtCK, from human and chicken. Using surface plasmon resonance, gel filtration, and enzyme assays, we have quantified properties that are of basic importance for MtCK functioning in vivo: membrane binding, octameric state, and enzymatic activity. Anthracyclines significantly impaired all three properties with differences in dose-, time-, and drug-dependence. Membrane binding and enzymatic activity were already affected at low anthracycline concentrations (5-100 microM), indicating high clinical relevance. Effects on membrane binding were immediate, probably because of competitive binding of the drug to cardiolipin. In contrast, dissociation of MtCK octamers into dimers, enzymatic inactivation and cross-linking occurred only after hours to days. Different protection assays suggest that the deleterious effects were caused by oxidative damage, mainly affecting the highly susceptible MtCK cysteines, followed by generation of free oxygen radicals at higher drug concentrations. Enzymatic inactivation occurred mainly at the active site and involved Cys278, as indicated by experiments with protective agents and sMtCK mutant C278G. All anthracycline effects were significantly more pronounced for sMtCK than for uMtCK. These in vitro results suggest that sMtCK damage may play a role in anthracycline cardiotoxicity. (+info)

Expression of creatine kinase isoenzyme genes during postnatal development of rat brain cerebellum: evidence for transcriptional regulation. (5/49)

Transcription and accumulation of brain-type creatine kinase (CKB) mRNA and its protein was examined during postnatal development of rat brain cerebellum, the brain region containing highest CKB mRNA in the adult. CKB protein was extremely low at day 1, increased about 10-fold until week 4 and remained constant until week 10. This time course was paralleled by cerebellar CKB mRNA, which was also extremely low at day 1 and increased 5-fold during the first 3 weeks and then remained constant. High levels of CKB protein were also detected in cultured primary cerebellar granular neurons. Nuclear run-on assays directly showed that CKB mRNA accumulation during postnatal cerebellar development was due to increased transcription. When compared with cerebrum and whole brain, cerebellar CKB mRNA accumulation during postnatal development was temporally delayed. Analysis of myocyte enhancer factor (MEF)-2 and Sp1, factors known to initiate or sustain CKB transcription in tissues other than brain, revealed that MEF-2 in cerebellum was low at week 1 but increased 3.5-fold by week 7, while Sp1 remained unchanged. The increase in CKB protein during cerebellar postnatal development was coincident with that of the ubiquitous mitochondrial CK protein and mRNA, indicating that a functional phosphocreatine energy shuttle probably exists for efficient ATP regeneration in the cerebellum. This should be beneficial for the many energy-demanding requirements during cerebellar development, as indicated by the observed temporal co-expression of CKB with myelin basic protein, which is involved in axon myelination by oligodendrocytes. (+info)

Mitochondrial creatine kinase is critically necessary for normal myocardial high-energy phosphate metabolism. (6/49)

The individual functional significance of the various creatine kinase (CK) isoenzymes for myocardial energy homeostasis is poorly understood. Whereas transgenic hearts lacking the M subunit of CK (M-CK) show unaltered cardiac energetics and left ventricular (LV) performance, deletion of M-CK in combination with loss of sarcomeric mitochondrial CK (ScCKmit) leads to significant alterations in myocardial high-energy phosphate metabolites. To address the question as to whether this alteration is due to a decrease in total CK activity below a critical threshold or due to the specific loss of ScCKmit, we studied isolated perfused hearts with selective loss of ScCKmit (ScCKmit(-/-), remaining total CK activity approximately 70%) using (31)P NMR spectroscopy at two different workloads. LV performance in ScCKmit(-/-) hearts (n = 11) was similar compared with wild-type hearts (n = 9). Phosphocreatine/ATP, however, was significantly reduced in ScCKmit(-/-) compared with wild-type hearts (1.02 +/- 0.05 vs. 1.54 +/- 0.07, P < 0.05). In parallel, free [ADP] was higher (144 +/- 11 vs. 67 +/- 7 microM, P < 0.01) and free energy release for ATP hydrolysis (DeltaG(ATP)) was lower (-55.8 +/- 0.5 vs. -58.5 +/- 0.5 kJ/mol, P < 0.01) in ScCKmit(-/-) compared with wild-type hearts. These results demonstrate that M- and B-CK containing isoenzymes are unable to fully substitute for the loss of ScCKmit. We conclude that ScCKmit, in contrast to M-CK, is critically necessary to maintain normal high-energy phosphate metabolite levels in the heart. (+info)

Differential effects of peroxynitrite on human mitochondrial creatine kinase isoenzymes. Inactivation, octamer destabilization, and identification of involved residues. (7/49)

Creatine kinase isoenzymes are very susceptible to free radical damage and are inactivated by superoxide radicals and peroxynitrite. In this study, we have analyzed the effects of peroxynitrite on enzymatic activity and octamer stability of the two human mitochondrial isoenzymes (ubiquitous mitochondrial creatine kinase (uMtCK) and sarcomeric mitochondrial creatine kinase (sMtCK)), as well as of chicken sMtCK, and identified the involved residues. Inactivation by peroxynitrite was concentration-dependent and similar for both types of MtCK isoenzymes. Because peroxynitrite did not lower the residual activity of a sMtCK mutant missing the active site cysteine (C278G), oxidation of this residue is sufficient to explain MtCK inactivation. Mass spectrometric analysis confirmed oxidation of Cys-278 and further revealed oxidation of the C-terminal Cys-358, possibly involved in MtCK/membrane interaction. Peroxynitrite also led to concentration-dependent dissociation of MtCK octamers into dimers. In this study, ubiquitous uMtCK was much more stable than sarcomeric sMtCK. Mass spectrometric analysis revealed chemical modifications in peptide Gly-263-Arg-271 located at the dimer/dimer interface, including oxidation of Met-267 and nitration of Trp-268 and/or Trp-264, the latter being a very critical residue for octamer stability. These data demonstrate that peroxynitrite affects the octameric state of MtCK and confirms human sMtCK as the generally more susceptible isoenzyme. The results provide a molecular explanation of how oxidative damage can lead to inactivation and decreased octamer/dimer ratio of MtCK, as seen in neurodegenerative diseases and heart pathology, respectively. (+info)

Protective effect of creatine against inhibition by methylglyoxal of mitochondrial respiration of cardiac cells. (8/49)

Previous publications from our laboratory have shown that methylglyoxal inhibits mitochondrial respiration of malignant and cardiac cells, but it has no effect on mitochondrial respiration of other normal cells [Biswas, Ray, Misra, Dutta and Ray (1997) Biochem. J. 323, 343-348; Ray, Biswas and Ray (1997) Mol. Cell. Biochem. 171, 95-103]. However, this inhibitory effect of methylglyoxal is not significant in cardiac tissue slices. Moreover, post-mitochondrial supernatant (PMS) of cardiac cells could almost completely protect the mitochondrial respiration against the inhibitory effect of methylglyoxal. A systematic search indicated that creatine present in cardiac cells is responsible for this protective effect. Glutathione has also some protective effect. However, creatine phosphate, creatinine, urea, glutathione disulphide and beta-mercaptoethanol have no protective effect. The inhibitory and protective effects of methylglyoxal and creatine respectively on cardiac mitochondrial respiration were studied with various concentrations of both methylglyoxal and creatine. Interestingly, neither creatine nor glutathione have any protective effect on the inhibition by methylglyoxal on the mitochondrial respiration of Ehrlich ascites carcinoma cells. The creatine and glutathione contents of several PMS, which were tested for the possible protective effect, were measured. The activities of two important enzymes, namely glyoxalase I and creatine kinase, which act upon glutathione plus methylglyoxal and creatine respectively, were also measured in different PMS. Whether mitochondrial creatine kinase had any role in the protective effect of creatine had also been investigated using 1-fluoro-2,4-dinitrobenzene, an inhibitor of creatine kinase. The differential effect of creatine on mitochondria of cardiac and malignant cells has been discussed with reference to the therapeutic potential of methylglyoxal. (+info)

Macro-CK type 2 is formed from mitochondrial CK polymer. Macro-CK type 1 has been associated with autoimmune and other chronic ... Macro-CK type 1 is a complex formed by one of the creatine kinase isoenzyme types, typically CK-BB, and antibodies; typically ... Macro-creatine kinase (macro-CK) is a macroenzyme, an enzyme of high molecular weight and prolonged half-life found in human ... Zhang, L; Han, F; Liu, X; Xie, C; Tian, K; Bi, Q; Hao, M; Mu, X (6 August 2020). "Macro creatine kinase in an asymptomatic ...

https://en.wikipedia.org/wiki/Macro-creatine_kinase

Fritz-Wolf K, Schnyder T, Wallimann T, Kabsch W (May 1996). "Structure of mitochondrial creatine kinase". Nature. 381 (6580): ... There are at least four different, but very closely related, forms of CK. Two isozymes, M (muscle) and B (brain), are cytosolic ... Creatine kinase (EC 2.7.3.2) (CK), which catalyses the reversible transfer of high energy phosphate from ATP to creatine, ... "Separate nuclear genes encode sarcomere-specific and ubiquitous human mitochondrial creatine kinase isoenzymes". J. Biol. Chem ...

https://en.wikipedia.org/wiki/ATP:guanido_phosphotransferase_family

Mitochondrial creatine kinase occurs in two different oligomeric forms: dimers and octamers, in contrast to the exclusively ... Ubiquitous mitochondrial creatine kinase has 80% homology with the coding exons of sarcomeric mitochondrial creatine kinase. ... Creatine kinase, mitochondrial 1B also known as CKMT1B is one of two genes which encode the ubiquitous mitochondrial creatine ... Schlattner U, Dolder M, Wallimann T, Tokarska-Schlattner M (2002). "Mitochondrial creatine kinase and mitochondrial outer ...

https://en.wikipedia.org/wiki/CKMT1B

Creatine+Kinase,+Mitochondrial+Form at the US National Library of Medicine Medical Subject Headings (MeSH) This article ... Creatine kinase U-type, mitochondrial, also called ubiquitous mitochondrial creatine kinase (uMtCK), is in humans encoded by ... "ASB9 interacts with ubiquitous mitochondrial creatine kinase and inhibits mitochondrial function". BMC Biology. 8: 23. doi: ... Schlattner U, Tokarska-Schlattner M, Wallimann T (February 2006). "Mitochondrial creatine kinase in human health and disease". ...

https://en.wikipedia.org/wiki/CKMT1A

Mitochondrial creatine kinase occurs in two different oligomeric forms: dimers and octamers, in contrast to the exclusively ... Creatine kinase S-type, mitochondrial is an enzyme that in humans is encoded by the CKMT2 gene. Mitochondrial creatine kinase ( ... Sarcomeric mitochondrial creatine kinase has 80% homology with the coding exons of ubiquitous mitochondrial creatine kinase. ... "Entrez Gene: CKMT2 creatine kinase, mitochondrial 2 (sarcomeric)". Human CKMT2 genome location and CKMT2 gene details page in ...

https://en.wikipedia.org/wiki/CKMT2

... there are two mitochondrial creatine kinase isoenzymes, the ubiquitous form and the sarcomeric form. The functional entity of ... While mitochondrial creatine kinase is directly involved in the formation of phosphocreatine from mitochondrial ATP, cytosolic ... Mitochondrial creatine kinase (CKm) is present in the mitochondrial intermembrane space, where it regenerates phosphocreatine ( ... Creatine kinase in the blood may be high in health and disease. Exercise increases the outflow of creatine kinase to the blood ...

https://en.wikipedia.org/wiki/Creatine_kinase

"In vitro complex formation between the octamer of mitochondrial creatine kinase and porin". The Journal of Biological Chemistry ... VDACs can also oligomerize to form part of the mitochondrial permeability transition pore (MPTP) and, thus, facilitate ... It forms an ion channel in the outer mitochondrial membrane (OMM) and also the outer cell membrane. In the OMM, it allows ATP ... Baines CP, Song CX, Zheng YT, Wang GW, Zhang J, Wang OL, Guo Y, Bolli R, Cardwell EM, Ping P (May 2003). "Protein kinase ...

https://en.wikipedia.org/wiki/VDAC1

Schlattner U, Tokarska-Schlattner M, Wallimann T (2006). "Mitochondrial creatine kinase in human health and disease". ... A 70 kg man contains around 120 g of creatine, with 40% being the unphosphorylated form and 60% as creatine phosphate. Of that ... is catalyzed by several creatine kinases. The presence of creatine kinase (CK-MB, creatine kinase myocardial band) in blood ... Phosphocreatine, also known as creatine phosphate (CP) or PCr (Pcr), is a phosphorylated form of creatine that serves as a ...

https://en.wikipedia.org/wiki/Phosphocreatine

The ATP-dependent cytosolic enzymes hexokinase, glucokinase, and glycerol kinase, as well as the mitochondrial enzyme creatine ... This protein contains about 280 amino acids and forms a beta barrel which spans the mitochondrial outer membrane. Since its ... This major protein of the outer mitochondrial membrane of eukaryotes forms a voltage-dependent anion-selective channel (VDAC) ... Voltage-dependent anion channels, or mitochondrial porins, are a class of porin ion channel located on the outer mitochondrial ...

https://en.wikipedia.org/wiki/Voltage-dependent_anion_channel

... and high levels of lactate and creatine kinase. The parents were found to be heterozygous carriers for the mutation. A third ... Mutations in NDUFAF1 can result in mitochondrial deficiencies and associated disorders. A disorder of the mitochondrial ... and some forms of Parkinson disease. In a patient with missense mutations in NDUFAF1, fatal infantile hypertrophic ... Wu L, Peng J, Ma Y, He F, Deng X, Wang G, Lifen Y, Yin F (2016). "Leukodystrophy associated with mitochondrial complex I ...

https://en.wikipedia.org/wiki/NDUFAF1

The reactions catalyzed by cytosolic (soluble) and mitochondrial GPDH are as follows: There are two forms of GPDH: The ... substrate pages: glycerol 3-phosphate, dihydroxyacetone phosphate related topics: glycerol phosphate shuttle, creatine kinase, ... In conjunction, Mitochondrial GPDH, or GPD2, is embedded on the outer surface of the inner mitochondrial membrane, overlooking ... The mitochondrial isoform of G3P dehydrogenase is thought to be inhibited by metformin, a first line drug for type 2 diabetes. ...

https://en.wikipedia.org/wiki/Glycerol-3-phosphate_dehydrogenase

... releasing intracellular muscle content into the blood as reflected by elevated blood levels of creatine kinase. Exercise ... The mitochondrial respiratory chain complex III catalyses electron transfer to cytochrome c. Complex III is embedded in the ... Chronic pain that makes a person unwilling to undertake a physical activity is not, by itself, a form of exercise intolerance. ... Mitochondrial complex III: Currently it is suggested that there are 27 different mutations identified in cytochrome b ( ...

https://en.wikipedia.org/wiki/Exercise_intolerance

EMG is either normal or may show myopathic low amplitude and short motor unit's potential (MUAPS). The enzymes creatine kinase ... This may occur as a result of reduction in muscle mitochondrial oxidative capacity and beta-adrenergic receptors, as well as ... The absence of painful spasms and pseudomyotonia differentiates this syndrome from its adult form, which is Hoffmann syndrome. ...

https://en.wikipedia.org/wiki/Kocher–Debre–Semelaigne_syndrome

... additionally also serum creatine kinase may be mildly above normal. Other exams/methods to ascertain if the individual has ... Ullrich congenital muscular dystrophy is a form of congenital muscular dystrophy. It is associated with variants of type VI ... Bernardi, Paolo; Bonaldo, Paolo (2013-05-01). "Mitochondrial Dysfunction and Defective Autophagy in the Pathogenesis of ... a cellular study of mitochondrial dysfunction and its rescue" (PDF). Brain. 132 (1): 147-155. doi:10.1093/brain/awn289. PMID ...

https://en.wikipedia.org/wiki/Ullrich_congenital_muscular_dystrophy

Creatine kinase (CK) blood level is often ordered when muscle damage is suspected. CK is an enzyme found in muscle, leaking ... Another form of operative scapular fixation is scapulopexy. "Scapulo-" refers to the scapula bone, and "-pexy" is derived from ... mitochondrial myopathy, Pompe disease, and polymyositis. Calpainopathy and scapuloperoneal myopathy, like FSHD, present with ... Casein kinase 1 (CK1) inhibition has been identified by Facio Therapies, a Dutch pharmaceutical company, to repress DUX4 ...

https://en.wikipedia.org/wiki/Facioscapulohumeral_muscular_dystrophy

After creation of the dTMP molecule, another kinase, thymidylate kinase, can act upon dTMP to create the diphosphate form, dTDP ... Patients with mutations in the thymidine kinase gene may have a certain type of mitochondrial DNA depletion syndrome, a disease ... Other small molecules that are substrates of kinases include creatine, phosphoglycerate, riboflavin, dihydroxyacetone, ... Kinases are classified into broad groups by the substrate they act upon: protein kinases, lipid kinases, carbohydrate kinases. ...

https://en.wikipedia.org/wiki/Kinase

... resource is short lasting because oxygen is required for the resynthesis of phosphocreatine via mitochondrial creatine kinase. ... lifestyle intervention and specific forms of exercise to rehabilitate and manage acute and chronic injuries and conditions. ... but the most readily depleted of the above sources is the PCr system which utilizes the enzyme creatine kinase. This enzyme ... The quick energy sources consist of the phosphocreatine (PCr) system, fast glycolysis, and adenylate kinase. All of these ...

https://en.wikipedia.org/wiki/Exercise_physiology

Blood tests show a creatine kinase activity greater than 1,000 U/L, with severe disease being above 5,000-15,000 U/L. The ... Milder forms may not cause any muscle symptoms, and the diagnosis is based on abnormal blood tests in the context of other ... For instance, mitochondrial diseases are characterized by ragged red fibers. Biopsy sites may be identified by medical imaging ... The most reliable test in the diagnosis of rhabdomyolysis is the level of creatine kinase (CK) in the blood. This enzyme is ...

https://en.wikipedia.org/wiki/Rhabdomyolysis

... itself can be phosphorylated by creatine kinase to form phosphocreatine, which is used as an energy buffer in skeletal ... Creatine's impact on mitochondrial function has led to research on its efficacy and safety for slowing Parkinson's disease. As ... A cyclic form of creatine, called creatinine, exists in equilibrium with its tautomer and with creatine. Creatine is ... Persky AM, Rawson ES (2007). "Safety of creatine supplementation". Creatine and Creatine Kinase in Health and Disease. ...

https://en.wikipedia.org/wiki/Creatine

Tests can be run to check creatine kinase in the blood, which is often normal or mildly elevated in congenital myopathies. ... In its severest form, affected babies often die from respiratory failure. To date, 9 gene mutations have been found to cause ... Cylindrical spirals have also been shown to react with the mitochondrial enzyme succinate dehydrogenase, which suggests that ... Diagnosis usually relies on this method, as creatine kinase levels and electromyography can be unreliable and non-specific. ...

https://en.wikipedia.org/wiki/Congenital_myopathy

... creatine kinase), kidney damage (uric acid), and stress damage (cortisol). Their findings suggested that high levels of plasma ... They found evidence that the supplementation positively affects mitochondrial deficiency syndrome and the symptoms of aging. ... the reduced form of Coenzyme Q10, produced favorable responses in children with autism. Professors Crane, Navas, and ... a 2-year treatment with Coenzyme Q10 in the ubiquinone form (3 times 100 mg/day) demonstrated significant improvement in the ...

https://en.wikipedia.org/wiki/Plácido_Navas_Lloret

In fact, there is evidence that VDAC binding by the anti-apoptotic HK1 and by the pro-apoptotic creatine kinase are mutually ... As one of two mitochondrial isoforms of hexokinase and a member of the sugar kinase family, HK1 catalyzes the rate-limiting and ... In the prefrontal cortex, HK1 putatively forms a protein complex with EAAT2, Na+/K+ ATPase, and aconitase, which functions to ... Activation of Akt kinase is mediated by HK1-VDAC1 coupling as part of the growth factor-mediated phosphatidyl inositol 3-kinase ...

https://en.wikipedia.org/wiki/HK1

Creatine kinase (CPK-MM) levels in the bloodstream are extremely high. An electromyography (EMG) shows that weakness is caused ... Form-fitting removable leg braces that hold the ankle in place during sleep can defer the onset of contractures. Appropriate ... "Duchenne Muscular Dystrophy: Pathophysiological Implications of Mitochondrial Calcium Signaling and ROS Production". 2 May 2012 ... Those affected also have a high level of creatine kinase in their blood. Although there is no known cure, physical therapy, ...

https://en.wikipedia.org/wiki/Duchenne_muscular_dystrophy

... a serine/threonine-specific protein kinase. The free acid (HMB-FA) and monohydrated calcium salt (HMB-Ca) forms of HMB have ... muscle enzymes such as creatine kinase and lactate dehydrogenase) in humans following intense exercise may be due to a ... α-KIC is mostly metabolized by the mitochondrial enzyme branched-chain α-ketoacid dehydrogenase, which converts it to ... HMB is sold as an over-the-counter dietary supplement in the free acid form, β-hydroxy β-methylbutyric acid (HMB-FA), and as a ...

https://en.wikipedia.org/wiki/Beta-Hydroxy_beta-methylbutyric_acid

Adult patients often have serum and/or urine screen positive for the presence of myoglobin and serum creatine kinase and ... Severe forms may have continual pain from general life activity. The adult form has a variable age of onset. The first ... It has been proposed that this segment mediates the association of CPT II with the inner mitochondrial membrane. Moreover, the ... This is noteworthy in light of the fact that this mutation is associated with the exercise-induced adult form (i.e., rising ...

https://en.wikipedia.org/wiki/Carnitine_palmitoyltransferase_II_deficiency

Zong H, Ren JM, Young LH, Pypaert M, Mu J, Birnbaum MJ, Shulman GI (December 2002). "AMP kinase is required for mitochondrial ... protein kinase A). AMPK is a heterotrimeric protein complex that is formed by α, β, and γ subunits. Each of these three ... expression in skeletal muscle in response to creatine depletion. PGC-1α is a transcriptional regulator for genes involved in ... liver kinase B1 (LKB1), which works in a complex with STRAD and MO25, Calcium/calmodulin-dependent protein kinase kinase II-( ...

https://en.wikipedia.org/wiki/AMP-activated_protein_kinase

... creatine kinase, mb form MeSH D08.811.913.696.640.150.750 - creatine kinase, mitochondrial form MeSH D08.811.913.696.640.150. ... arginine kinase MeSH D08.811.913.696.640.150 - creatine kinase MeSH D08.811.913.696.640.150.500 - creatine kinase, bb form MeSH ... map kinase kinase kinase 1 MeSH D08.811.913.696.620.682.700.559.200 - map kinase kinase kinase 2 MeSH D08.811.913.696.620.682. ... map kinase kinase kinase 3 MeSH D08.811.913.696.620.682.700.559.400 - map kinase kinase kinase 4 MeSH D08.811.913.696.620.682. ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D08)

... elevated serum creatine kinase levels, and white matter involvement". American Journal of Medical Genetics. Part A. 161A (12): ... The k-nearest neighbors algorithm predicts C3orf62 to be classified as follows: k=9/23; 69.6% nuclear, 13.0% mitochondrial, ... Transcription of C3orf62 produces 5 alternatively spliced variants and 1 unspliced form. Of the four splice variants, two of ... No ortholog sequences of C3orf62 were found for the following life forms: Bacteria, archaea, protist, plant, fungus, trichoplax ...

https://en.wikipedia.org/wiki/C3orf62

... of energy in the form of creatine phosphate which is generated from ATP and can regenerate ATP when needed with creatine kinase ... This would be brought about by an increase in mitochondrial size and number and the associated related changes, not a change in ... Each somite has three divisions, sclerotome (which forms vertebrae), dermatome (which forms skin), and myotome (which forms ... Thus-through functional genomics-calcineurin, calmodulin-dependent kinase, PGC-1α, and activated PPARδ form the basis of a ...

https://en.wikipedia.org/wiki/Skeletal_muscle

It is possible that aberrant immune activity during critical periods of neurodevelopment is part of the mechanism of some forms ... Mitochondrial DNA Mutations Nuclear DNA mutations Biotinidase deficiency Urea cycle defects Epigenetic mechanisms may increase ... glycine amidinotransferase deficiency Guanidinoacetate methyltransferase deficiency X-linked creatine transporter defect 6-N- ... Homocystinuria Branched-chain ketoacid dehydrogenase kinase deficiency Succinic semialdehyde dehydrogenase deficiency Smith- ...

https://en.wikipedia.org/wiki/Causes_of_autism

This combination results in either an ADP-forming succinate-CoA ligase (A-SUCL, EC 6.2.1.5) or a GDP-forming succinate-CoA ... Chinopoulos, C (2011). "Mitochondrial consumption of cytosolic ATP: not so fast". FEBS Lett. 585 (9): 1255-9. doi:10.1016/j. ... 1,3-bisphosphoglycerate is then dephosphorylated via phosphoglycerate kinase, producing 3-phosphoglycerate and ATP through a ... and the enzyme creatine phosphokinase transfers a phosphate from phosphocreatine to ADP to produce ATP. Then the ATP releases ...

https://en.wikipedia.org/wiki/Substrate-level_phosphorylation

... myopathic form; 609560; TK2 Mitochondrial DNA-depletion syndrome, hepatocerebral form; 251880; DGUOK Mitochondrial myopathy and ... L1CAM Creatine deficiency syndrome, X-linked; 300352; SLC6A8 Creatine phosphokinase, elevated serum; 123320; CAV3 Creutzfeldt- ... response to tyrosine kinase inhibitor in; 211980; EGFR Nonsmall cell lung cancer, somatic; 211980; IRF1 Nonsmall cell lung ... hepatocerebral form; 251880; C10orf2 Mitochondrial DNA depletion syndrome, hepatocerebral form; 251880; MPV17 Mitochondrial DNA ...

https://en.wikipedia.org/wiki/List_of_OMIM_disorder_codes

... include muscle pain and weakness in the presence of an elevated creatine kinase (CK). King brown snake venom has some ... If the smoke resembled the mali, or immaterial form, of a snake, then the person would die, as the victim of a ragalk (sorcerer ... In 2017, British herpetologist Simon Maddock and colleagues published a genetic analysis using mitochondrial DNA on the genus, ... Clade II corresponded to a lineage of large snakes found across Australia, clade III was a dwarf form from the Kimberley, and ...

https://en.wikipedia.org/wiki/King_brown_snake

Khan HA, Alhomida AS, Sobki SH, Moghairi AA, Koronki HE (2012). "Blood cell counts and their correlation with creatine kinase ... They form an essential part of the innate immune system, with their functions varying in different animals. They are formed ... "Mitochondrial calcium uniporter affects neutrophil bactericidal activity during Staphylococcus aureus infection". Infection and ... Trapping of bacteria may be a particularly important role for NETs in sepsis, where NETs are formed within blood vessels. ...

https://en.wikipedia.org/wiki/Neutrophil

In a similar mechanism, the pro-apoptotic creatine kinase binds and opens VDAC in the absence of HK2. An alternative model ... "Mitochondrial hexokinase II promotes neuronal survival and acts downstream of glycogen synthase kinase-3". The Journal of ... This gene encodes a 100-kDa, 917-residue enzyme with highly similar N- and C-terminal domains that each form half of the ... Okatsu K, Iemura S, Koyano F, Go E, Kimura M, Natsume T, Tanaka K, Matsuda N (Nov 2012). "Mitochondrial hexokinase HKI is a ...

https://en.wikipedia.org/wiki/HK2

Common clinical manifestations of MDCMC include: early-onset hypotonia muscle wasting mildly elevated serum creatine kinase (CK ... The structure of the protein has been found to be a homodimer, and forms a heterodimer with the CHKA protein. It has also been ... Mutations in CHKB have been found to result in mitochondrial deficiencies and associated disorders. Knockdown of the gene has ... Choline kinase beta (CK), also known as Ethanolamine kinase (EK), Choline kinase-like protein , choline/ethanolamine kinase ...

https://en.wikipedia.org/wiki/CHKB_(gene)

... and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The ... This enzyme is also a tetramer formed by two dimers, and the monomers and dimers are assembled in a manner that allows the ... Ornithine and/or arginine are key intermediates for the synthesis of urea, creatine, nitric oxide, polyamines, and protein; ... The gamma-glutamyl kinase domain employs a 367-residue chain that folds into an N-terminal amino acid kinase domain, ...

https://en.wikipedia.org/wiki/Aldehyde_dehydrogenase_18_family,_member_A1

As a result, nitrogen (in the form of ammonia) and other byproducts of the urea cycle (such as citrulline) build up in the ... The activity of argininosuccinate synthetase in arginine synthesis occurs largely in at the outer mitochondrial membrane of ... Haines RJ, Corbin KD, Pendleton LC, Eichler DC (July 2012). "Protein kinase Cα phosphorylates a novel argininosuccinate ... Argininosuccinate synthetase is involved in the synthesis of creatine, polyamines, arginine, urea, and nitric oxide. The ...

https://en.wikipedia.org/wiki/Argininosuccinate_synthase

"Creatine kinase". GPnotebook. Page 585 in: Lee, Mary Ann (2009). Basic Skills in Interpreting Laboratory Data. Amer Soc of ... 2009 Derived form molar values using molar mass of 1355g/mol Derived from mass values using molar mass of 1355g/mol " ... Mitochondrial Antibodies (M2), Serum". www.mayocliniclabs.com. Mayo Clinic Laboratories. Retrieved 2 July 2020. Rajkumar SV, ...

https://en.wikipedia.org/wiki/Reference_ranges_for_blood_tests

Mutant huntingtin protein has been found to play a key role in mitochondrial dysfunction. The impairment of mitochondrial ... This altered form, called mutant huntingtin (mHtt), increases the decay rate of certain types of neurons. Regions of the brain ... These include chorea acanthocytosis and pantothenate kinase-associated neurodegeneration. One X-linked disorder of this type is ... creatine, minocycline, ethyl-EPA, phenylbutyrate and dimebon. Medicine portal Dayalu P, Albin RL (February 2015). "Huntington ...

https://en.wikipedia.org/wiki/Huntington's_disease

... serum creatine kinase, serum lactate dehydrogenase, urinary urea nitrogen, and urinary 3-methylhistidine. When exercise ... Supplements are sold either as single ingredient preparations or in the form of "stacks" - proprietary blends of various ... mitochondrial dynamics and functions). Moreover, HMB is cheap (~30- 50 US dollars per month at 3 g per day) and may prevent ... Some studies have suggested that consumption of creatine with protein and carbohydrates can have a greater effect than creatine ...

https://en.wikipedia.org/wiki/Bodybuilding_supplement

Among them creatine, Coenzyme Q10 and mitochondrial targeted antioxidants/peptides are reported to have the most remarkable ... Protein kinase B (Akt) is thought to downregulate PGC-1α, but upregulate its downstream effectors, NRF1 and NRF2. Akt itself is ... both formed endogenously in the cell as by-products of metabolism but upregulated during times of cellular stress. Fasting can ... Mitochondrial biogenesis is therefore defined as the process via which cells increase their individual mitochondrial mass [3 ...

https://en.wikipedia.org/wiki/PPARGC1A

AST exists in two isoenzymes namely mitochondrial form and cytoplasmic form. It is found in highest concentration in the liver ... Nageh T, Sherwood RA, Harris BM, Byrne JA, Thomas MR (2003). "Cardiac troponin T and I and creatine kinase-MB as markers of ... More than 80% of the liver AST activity are contributed by mitochondrial form of the isoenzymes, while the circulating AST in ... LDH isotype-1 (or cardiac) is used for estimating damage to cardiac tissue, although troponin and creatine kinase tests are ...

https://en.wikipedia.org/wiki/Liver_function_tests

BCAA supplementation has been shown to decrease levels of creatine kinase in muscle cells post exercise. Creatine kinase is an ... Isoleucine forms a negative feedback loop with threonine dehydrogenase. Acetohydroxyacid synthase is the first enzyme for the ... "Branched-chain amino acid supplementation promotes survival and supports cardiac and skeletal muscle mitochondrial biogenesis ... BCAA supplementation has been shown to decrease levels of creatine kinase, leading to higher levels of intracellular ATP and a ...

https://en.wikipedia.org/wiki/Branched-chain_amino_acid

Tyrosine Phosphorylation of Mitochondrial Creatine Kinase 1 Enhances a Druggable Tumor Energy Shuttle Pathway. Kurmi, K., ... Glycogen synthase kinase 3 (GSK-3)-mediated phosphorylation of uracil N-glycosylase 2 (UNG2) facilitates the repair of ... Enzymatic activation of pyruvate kinase increases cytosolic oxaloacetate to inhibit the Warburg effect. Wiese, E. K., Hitosugi ... Effects of selective checkpoint kinase 1 inhibition on cytarabine cytotoxicity in acute myelogenous leukemia cells in vitro. ...

https://mayoclinic.pure.elsevier.com/en/persons/larry-m-karnitz/publications/?page=1&ordering=publicationYearThenTitle&descending=false

E5.200.812.735.645.350.350.150 Creatine Kinase, Mitochondrial Form D12.776.575.186 Crossing Over, Genetic G5.355.760.210 G5.355 ... A-Form G2.111.570.790.486.128 G5.360.580.114 DNA, C-Form G2.111.570.790.486.156 G5.360.580.128 DNA, Catenated G2.111.570.790. ... D9.408.477.500 Mitochondrial ADP, ATP Translocases D12.776.543.585.475.500 Mitochondrial Proton-Translocating ATPases D12.776. ... D12.776.476.150.299.300 Casein Kinase II D12.644.360.150.600 Casein Kinases D12.644.360.150 CD4 Lymphocyte Count E1.450.375.107 ...

https://decs.bvs.br/P/mnchg2012.txt

Creatine Kinase, Mitochondrial Form Entry term(s). Creatine Kinase, Mitochondrial Kinase, Mitochondrial Creatine Mitochondrial ... Mitochondrial creatine kinase Entry term(s):. Creatine Kinase, Mitochondrial. Kinase, Mitochondrial Creatine. Mitochondrial ... Creatine Kinase, Mitochondrial Form - Preferred Concept UI. M0363590. Scope note. A form of creatine kinase found in the ... 2006; CREATINE KINASE, MITOCHONDRIAL FORM was indexed under CREATINE KINASE 2000-2005. ...

https://decs.bvsalud.org/en/ths/resource/?id=50744

... creatine kinase], porin and hexokinase at the cytosolic surface, in which nucleotides are channelled in and out the ... Structure and evolution of mitochondrial porin (VDAC) Schema for the mitochondrial pore. The porin molecule has been modeled as ... Hexokinase (HK) exists naturally as a soluble (yeast) or a mitochondria-bindable (hepatoma, mammalian brain) form. The binding ... Mitochondrial compartments potentially subject to ATP monitoring by differently localized firefly luciferase probes. The system ...

https://www.bgu.ac.il/~aflaloc/hilights.html

... the cytoplasmic muscle form; and CK-Mt2, the sarcomeric mitochondrial form. The protein expression and phosphorylation of these ... It catalyzes the reversible transfer of the γ-phosphate group from ATP to creatine to yield ADP and creatine phosphate. Because ... This study investigated three isoforms of CK: CK-B, the nonmuscle cytoplasmic form; CK-M, ... Creatine kinase (CK) is an essential phosphoryl transferase enzyme in cellular energy homeostasis. ...

https://scholarworks.smith.edu/theses/1610/

... in sharp contrast to the relatively high degree of conservation of intron positions seen in a homologous enzyme creatine kinase ... have what appear to be N-terminal mitochondrial targeting sequences, providing the first evidence for true mitochondrial AK ... inates (m/z 315.20) in its triply protonated form. The intensity. of the diphosphorylated peptide ions [represented by m/z ... A protein kinase was purified from rat liver nuclei by affinity chromatography on poly(l-lysine)-agarose and protein kinase ...

https://www.researchgate.net/publication/41418560_Electron_Capture_Dissociation_Mass_Spectrometric_Analysis_of_Lysine-Phosphorylated_Peptides

Creatine Kinase, BB Form. *Creatine Kinase, MB Form. *Creatine Kinase, Mitochondrial Form ... Macro-creatine kinase refers to creatine kinase complexed with other serum proteins. ... "Creatine Kinase" by people in this website by year, and whether "Creatine Kinase" was a major or minor topic of these ... "Creatine Kinase" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH (Medical Subject ...

https://profiles.umassmed.edu/display/125433

https://decs2017.bvsalud.org/E/mnchg2012.txt

https://decs2015.bvsalud.org/E/mnchg2012.txt

https://decs2013.bvsalud.org/P/mnchg2012.txt

https://decs2014.bvsalud.org/I/mnchg2012.txt

https://decs2020.bvsalud.org/E/mnchg2012.txt

https://decs2016.bvsalud.org/E/mnchg2012.txt

https://decs2019.bvsalud.org/E/mnchg2012.txt

https://decs2018.bvsalud.org/P/mnchg2012.txt

https://decs2012.bvsalud.org/P/mnchg2012.txt

Ubiquitous mitochondrial creatine kinase (uMtCK), a key enzyme in energy metabolism, was identified by differential display PCR ... Cyclocreatine (cCr), whose phosphorylated form is a very poor substrate for CK, inhibited proliferation of the L1236 cell line ... Hodgkin disease-derived cell lines expressing ubiquitous mitochondrial creatine kinase show growth inhibition by cyclocreatine ... This inhibition by cCr was partially reversed by competition with creatine, which by itself had no effect on proliferation of ...

https://pesquisa.bvsalud.org/brasil/?lang=pt&q=au:Staratschek-Jox,+A

Creatine kinases catalyze reversible transfer of phosphoryl groups between ATP and creatine, mainly in high energetic ... Mitochondrial epigenetics are in nascent form and should be better and extensively studied (Manev et al., 2012). ... Relevant functional parameters of energy metabolism, succinate dehydrogenase and creatine kinase activities, were also analyzed ... Iron induced mitochondrial DNA (mtDNA) deletions, decreased epigenetic modulation of mtDNA, mitochondrial ferritin levels, and ...

https://www.grecc.org/publications/ressources-documentaires/pharmacologie/novel-insights-into-mitochondrial-molecular-targets-of-iron-inducedneurodegeneration-reversal-by-cannabidiol-vanessa-kappel-da-silva-et-al-2018/

... regularly shaped inclusions highly enriched in creatine kinase (CK) form inside their mitochondria. The emergence of these ... The CK-rich mitochondrial inclusions are thought to be macromolecular complexes that form as a result of metabolic adaptation ... Zurmanova J, Difato F, Malacova D, Mejsnar J, Stefl B, Zahradnik I: Creatine kinase binds more firmly to the M-band of rabbit ... Mitochondrially-associated enzymes form a functional glycolytic sequence that supports mitochondrial respiration through ...

https://tbiomed.biomedcentral.com/articles/10.1186/1742-4682-6-6

Computational image analysis revealed only a single projection class of molecules, but the ability of Mi-CK to form linear ... Structure of the mitochondrial creatine kinase octamer: high-resolution shadowing and image averaging of single molecules and ... T Schnyder, H Gross, H Winkler, H M Eppenberger, T Wallimann; Structure of the mitochondrial creatine kinase octamer: high- ... with circular harmonic image averaging revealed details on the surface structure of mitochondrial creatine kinase (Mi-CK) ...

https://rup.silverchair.com/jcb/article-standard/112/1/95/13963/Structure-of-the-mitochondrial-creatine-kinase

... that of glutamine synthetase and creatine kinase, which are markedly reduced in AD brains (60), reflecting the alteration of ... one of the major forms of antioxidant defense consists of storing and transporting the iron in forms that do not catalyse the ... Turrens JF: Mitochondrial formation of reactive oxygen species. J Physiol. 552:335-344. 2003. View Article : Google Scholar : ... whereas oxidative impairment of creatine kinase may cause decreased energy metabolism in AD (61). The pathologic aggregation of ...

https://www.spandidos-publications.com/10.3892/br.2016.630

Creatine Kinase 34% * Basement Membrane 32% * Mitochondrial Membranes 18% * Actin Cytoskeleton 16% ... Together they form a unique fingerprint. ...

https://augusta.pure.elsevier.com/en/publications/correlative-studies-on-sarcolemmal-ultrastructure-permeability-an/fingerprints/

In muscle tissue, creatine generally occurs as phosphocreatine. Creatine is excreted as creatinine in the urine. ... forming creatine phosphate. The reaction is catalysed by creatine kinase, and the result is phosphocreatine (PCr). ... The chicken acidic type mitochondrial creatine kinase gene as the first nonmammalian gene. J Biol Chem. 1996 May 17;271(20): ... Stachowiak O, Dolder M, Wallimann T, Richter C: Mitochondrial creatine kinase is a prime target of peroxynitrite-induced ...

https://go.drugbank.com/drugs/DB00148

Rhabdomyolysis is defined as a serum creatine kinase level of more than ten times the upper limit of normal.1. Severe physical ... Myoglobin formed as the result of muscle injury is filtrated and causes direct renal injury by being reabsorbed from renal ... It was also hypothesized that fibrates only exacerbate latent preexisting mitochondrial myopathies or accelerate the normal ...

https://jpma.org.pk/article-details/3623

Aldehyde Dehydrogenase, Mitochondrial [D12.776.575.093] * Creatine Kinase, Mitochondrial Form [D12.776.575.186] * DNA ... Mitochondrial Membrane Transport Proteins [D12.776.575.750] * Mitochondrial ADP, ATP Translocases [D12.776.575.750.500] ... Mitochondrial Membrane Transport Proteins [D12.776.543.585.475] * Mitochondrial ADP, ATP Translocases [D12.776.543.585.475.500] ... Mitochondrial Permeability Transition Pore [D12.776.543.585.475.563] * Mitochondrial Precursor Protein Import Complex Proteins ...

https://meshb-prev.nlm.nih.gov/record/ui?ui=D033681

In this Review, we discuss the role of metabolic and mitochondrial pathways dysregulated in acute myeloid leukemia, and the ... have shown that mitochondrial function was specifically driven by the creatine kinase pathway in the EVI1 subgroup of patients ... and small molecules selectively inhibiting the mutated forms of these enzymes have been developed and very recently approved ... This also provides mitochondrial protection against mitochondrial death pathways by preventing opening of the mitochondrial ...

https://bmcbiol.biomedcentral.com/articles/10.1186/s12915-019-0670-4

... and serum creatine kinase isoenzyme (CK-MB) and cardiac troponin (CTnI) levels were measured. Finally, the relative mRNA and ... CTnl cardiac troponin, CK-MB creatine kinase isoenzyme, N normal, IR ischemia-reperfusion, IPO ischemia post-conditioning, DM ... When the mitochondrial self-storage breathing capacity disappears completely, a large amount of ROS is generated, resulting in ... Reactive oxygen species have a causal role in multiple forms of insulin resistance. Nature. 2006;440(7086):944-8. ...

https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/s12872-021-01949-z

found that leonurine increased to SOD activity by decreasing levels of lactate dehydrogenase (lDH), creatine kinase (CK), and ... Involvement of mitochondrial function and HIF-1α dependent VEGF activation Improve the ultrastructure of mitochondrion ... Cardiac hypertrophy is a powerful form of compensation, but it isnt infinite. If there isnt remission, the heart function may ... lDH=lactate Dehydrogenase; CK=Creatine Kinase; SOD=Superoxide Dismutase; MDA=Malondialdehyde; Bcl-2=B cell lymphoma-2; Bax=BCl2 ...

https://www.fortunejournals.com/articles/the-cardioprotective-effect-of-leonurine-in-hearts.html

Serum creatine kinase and urine myoglobin levels are markedly elevated. Clinical examination, history, laboratory studies, ... Dive into the research topics of Diagnostic evaluation of rhabdomyolysis. Together they form a unique fingerprint. ... and mitochondrial oxidative phosphorylation. Less common inherited causes of rhabdomyolysis include structural myopathies, ... Serum creatine kinase and urine myoglobin levels are markedly elevated. Clinical examination, history, laboratory studies, ...

https://jhu.pure.elsevier.com/en/publications/diagnostic-evaluation-of-rhabdomyolysis-4

After perfusion was performed according to the experimental strategy, the area of myocardial infarction (MI), and serum creatine kinase isoenzyme (CK-MB) and cardiac troponin (CTnI) levels were measured. (biomedcentral.com)
Serum creatine kinase and urine myoglobin levels are markedly elevated. (elsevier.com)
Serum creatine kinase (CK) is elevated in several cases but not all. (medscape.com)
In EDMD serum creatine of elbow flexors was 3/5, shoulder abducc kinase (CK) level is normal or moderately tors 3/5 and other muscles 4/5. (who.int)
Serum creatine kinase, vascular endothelial growth factor, interleukin-8 and granulocyte macrophage colony stimulating factor were determined on days 1, 7 and 14 after AMI. (biomedcentral.com)
Interestingly, pre- and post-treatment with Avemar or Echinacea substantially increased serum creatine kinase on day 1, normalized its activity on day 7 and, on continued treatment, only Echinacea markedly increased its activity on day 14 compared to the corresponding AMI values. (biomedcentral.com)
These include serum measurements of muscle necrosis parameters (serum creatine kinase, CK), electromyography (EMG) and nerve conduction studies, and muscle biopsy. (biomedcentral.com)

A form of creatine kinase found in the MITOCHONDRIA . (bvsalud.org)
Hexokinase (HK) exists naturally as a soluble (yeast) or a mitochondria-bindable (hepatoma, mammalian brain) form. (bgu.ac.il)
Thus, mitochondria function as both the source and target of toxic ROS since mitochondrial dysfunction and oxidative stress are important in aging and neurodegenerative diseases, particularly AD ( 11 , 12 ). (spandidos-publications.com)
Mitochondrial myopathy is caused by a defect in the mitochondria, which are the energy-producing part of cells. (clevelandclinic.org)

Three cytoplasmic ISOENZYMES have been identified in human tissues: the MM type from SKELETAL MUSCLE, the MB type from myocardial tissue and the BB type from nervous tissue as well as a mitochondrial isoenzyme. (umassmed.edu)
To identify patients with periprocedural MI (PMI), several conflicting definitions of PMI have been proposed, relying either on cardiac troponin (cTn) or the MB isoenzyme of creatine kinase, with or without supporting evidence of ischaemia. (medscape.com)

The most energy-dependent organs of the body, such as the brain, heart, skeletal muscles and endocrine pancreas, are commonly affected by mitochondrial disease as they are most vulnerable to the dysfunction of oxidative ATP production. (bmj.com)
This review will address the effects of ZIKV infection on CNS and PNS glial cells, focusing on cellular and molecular mechanisms, including changes in the inflammatory response, oxidative stress, mitochondrial dysfunction, Ca2+ and glutamate homeostasis, neural metabolism, and neuron-glia communication. (bvsalud.org)
Advances in understanding the tau protein aggregation and mitochondrial dysfunction of progressive supranuclear palsy and the toxic and genetic insults underlying them are accruing swiftly. (medlink.com)

Glutathione functions for resisting oxidative stress with the enzyme glutathione reductase (GR), recycling oxidized glutathione back to the reduced form. (zenithnutrition.com)

Macro-creatine kinase refers to creatine kinase complexed with other serum proteins. (umassmed.edu)
The uranyl ion forms complexes with bicarbonate, citrate, and other soluble anionic species, and binds to proteins in tissue and plasma. (cdc.gov)
Thus, we investigated the effects of sulfite on redox and mitochondrial homeostasis, as well as signaling proteins in the cerebral cortex of rat pups. (bvsalud.org)
Protein-SH groups are amongst the most easily oxidized residues in proteins, but irreversible oxidation can be prevented by protein glutathionylation, in which protein-SH groups form mixed disulphides with glutathione. (biomedcentral.com)

Iron induced mitochondrial DNA (mtDNA) deletions, decreased epigenetic modulation of mtDNA, mitochondrial ferritin levels, and succinate dehydrogenase activity. (grecc.org)
SDH, also known as succinate ubiquinone oxidoreductase or mitochondrial complex II, is the only multi-subunit enzyme integrated in the inner mitochondrial membrane of the tricarboxylic acid cycle (TCA). (biomedcentral.com)
Sulfite also reduced the activities of succinate dehydrogenase, creatine kinase, and respiratory chain complexes II and II-III. (bvsalud.org)

2016), decreased levels of synaptophysin, as well as alterations in the levels of DNM1L, a protein critically involved in mitochondrial fission (da Silva et al. (grecc.org)
Primary focus is on the multifunctional Ca2+ and calmodulin-dependent protein kinase II (CaMKII). (hopkinsmedicine.org)
This protein forms part of the contractile (sarcomeric) apparatus in the fast glycolytic fibers of human skeletal muscle-the fibers responsible for the generation of rapid, forceful contractions in activities such as sprinting and weightlifting-and is thought to perform specialized role(s) important to the function of these fibers. (lww.com)
Models have been proposed in which this protein modification does not require an enzymatic activity, but proceeds via the reaction of partially oxidised protein sulphydryls with GSH, or by thiol/disulphide exchange reactions with the oxidised disulphide form of glutathione (Fig. 1 ) [ 9 ]. (biomedcentral.com)

Inherited predisposition to rhabdomyolysis can occur with disorders of glycogen metabolism, fatty acid β-oxidation, and mitochondrial oxidative phosphorylation. (elsevier.com)
The regular active form of cysteine has low intestinal absorption, poor water solubility, and rapid hepatic metabolism. (zenithnutrition.com)
Defects of mitochondrial metabolism cause a wide range of human diseases. (iospress.com)

A transferase that catalyzes formation of PHOSPHOCREATINE from ATP + CREATINE. (umassmed.edu)
In muscle tissue, creatine generally occurs as phosphocreatine. (drugbank.com)
Most of the creatine is metabolized in these tissues to phosphocreatine (creatine phosphate). (drugbank.com)
Phosphocreatine is a major energy storage form in the body. (drugbank.com)
The reaction is catalysed by creatine kinase, and the result is phosphocreatine (PCr). (drugbank.com)
Role of the creatine/phosphocreatine system in the regulation of mitochondrial respiration. (naturalbodyinc.com)
Creatine supplementation: exploring the role of the creatine kinase/phosphocreatine system in human muscle. (naturalbodyinc.com)

2008). Mitochondrial DNA damage has already been related to aging and neurodegenerative diseases and contribute to mitochondrial disruption, which in turn may lead to cell injury, particularly in the central nervous system (CNS) (Siddiqui et al. (grecc.org)

Isolated mitochondrial myopathy is rare, but resembles genetic and acquired limb-girdle syndromes and Dok-7 congenital myasthenia. (bmj.com)

Contact sites between mitochondrial membranes are believed to harbor a multienzyme complex including matrix ATP synthase, translocase , [creatine kinase], porin and hexokinase at the cytosolic surface, in which nucleotides are channelled in and out the mitochondrion. (bgu.ac.il)
ATP5A1 ATP synthase, H+transporting, mitochondrial F1 complex, alpha subunit isoform bMiddleton et al. (h2sampk.com)

These conditions have muscle weakness, but also a variety of other symptoms, as mitochondrial disorders typically affect other organ systems like your heart, brain and gastrointestinal tract. (clevelandclinic.org)
Mitochondrial diseases are inherited disorders of oxidative phosphorylation that present with a multitude of clinical features in different combinations and with various inheritance patterns. (bmj.com)
To complicate the issue further, the clinical features of mitochondrial disorders overlap with common neurological and non-neurological diseases. (bmj.com)
Here, we briefly review some common clinical features associated with mitochondrial disease, and provide some clues as to how patients with these mitochondrial disorders might be identified. (bmj.com)
We discuss both 'chameleons'-mitochondrial disorders that may look like something else, and 'mimics'-other conditions that may clinically resemble mitochondrial disease. (bmj.com)
Mitochondrial disorders affect fewer than 1 in 5000 adults, 3 so there is only a low likelihood of seeing a new case in a general neurology clinic. (bmj.com)
Other forms of epileptic encephalopathy should be considered in the subacute setting, particularly if refractory to treatment (eg, epilepsy syndromes such as SCN4A gene-related, or other metabolic disorders such as glutaric aciduria type I). (bmj.com)
Treatment of mitochondrial disorders is a challenge, as there is only symptomatic therapy available. (iospress.com)
In this paper, we review the various treatments studied and practiced in the context of mitochondrial disorders. (iospress.com)

2013). A secondary aspect of creating resistance to aerobic fatigue in theory is the ability of it to spur mitochondrial respiration (Smith-Ryan et al. (seancochran.com)

Cardiac troponin, creatine phosphokinase, and lactate dehydrogenase levels were significantly increased after treatment in iNOS (-/-) mice as compared to controls and wildtype mice. (uky.edu)
NO levels mediated by ADR treatment leads to increased cardiac mitochondrial injury that can be attenuated by a compensatory increase in MnSOD. (uky.edu)

Diagnosis depends on the patient history and physical examination, muscle biopsy, electromyography, ischemic forearm test, and creatine kinase level. (medscape.com)

In this Review, we discuss the role of metabolic and mitochondrial pathways dysregulated in acute myeloid leukemia, and the potential of therapeutic intervention targeting these metabolic dependencies on the proliferation, differentiation, stem cell function and cell survival to improve patient stratification and outcomes. (biomedcentral.com)
One area of high interest and potential is targeting metabolic and mitochondrial pathways that are important in AML biology and that may constitute an Achilles heel of AML cells. (biomedcentral.com)
The clinical picture of late-onset forms is highly variable with symptoms ranging from acute metabolic decompensations to chronic, mainly muscular problems or even asymptomatic cases. (helmholtz-muenchen.de)

Mi-CK octamers exhibit a cross-like surface depression dividing the square shaped projection of 10 x 10 nm into four equally sized subdomains, which correspond to the four dimers forming the octameric Mi-CK molecule. (silverchair.com)
Computational image analysis revealed only a single projection class of molecules, but the ability of Mi-CK to form linear filaments, as well as geometrical considerations concerning the formation of octamers by four equal, asymmetric dimers, suggest the existence of at least two distinct faces on the molecule. (silverchair.com)
The general structure of the Mi-CK octamer described here is relevant to the localization of the molecule at the inner-outer mitochondrial contact sites and to the function of Mi-CK as an "energy channeling" molecule. (silverchair.com)
Pyrroloquinoline quinone (henceforth PQQ) is a small quinone molecule which has the ability to be a REDOX agent, capable of reducing oxidants (an antioxidant effect) and then being recycled by glutathione back into an active form. (pharmaceuticalintelligence.com)

and CK-Mt2, the sarcomeric mitochondrial form. (smith.edu)

The major role of creatine and PCr is to maintain ATP concentrations through the actions of the creatine kinase reaction (Haff et al. (seancochran.com)

The mitochondrial electron transport chain consumes almost 98% of molecular oxygen at the cytochrome oxidase complex and the remaining oxygen is reduced to hydrogen peroxide and superoxide radicals. (spandidos-publications.com)

HK1 Hexokinase brain form Prabakaran et al. (h2sampk.com)

Persons with Ullrich congenital muscular dystrophy, rigid spine with muscular dystrophy (deficiency of selenoprotein N), and integrin-α7 deficiency have creatine kinase (CK) levels that are normal to mildly elevated (≤5 times normal). (medscape.com)

Accordingly, since metal catalyzes redox reactions ( 3 ), one of the major forms of antioxidant defense consists of storing and transporting the iron in forms that do not catalyse the formation of reactive radicals ( 4 ), as is the case during tissue injury, in which there is an increase in iron availability that can accelerate free radical reactions ( 4 ). (spandidos-publications.com)

This limited evidence also suggests that the main claim of PQQ, an enhancement of mitochondrial function, occurs in otherwise healthy humans given PQQ supplementation. (pharmaceuticalintelligence.com)
The supplementation of creatine via a dietary supplement has demonstrated to increase creatine content of skeletal muscles (Haff et al. (seancochran.com)
1999). Additional studies show variances of these figures, though the supplementation presents increases in both free creatine and PCr (Haff et al. (seancochran.com)
Research indicates the supplementation of creatine has produced improvement in physical tests, created more optimal body composition, and improved blood markers such as reduced inflammatory indicators and decreased lactate (Machek & Bagley, 2018). (seancochran.com)
Creatine supplementation has shown to increase body mass. (seancochran.com)
2013). Relative to physiological benefits, research has suggested creatine supplementation having a positive effect on the lipid profile of middle-age males and females with elevated levels of triglycerides (Haff et al. (seancochran.com)
Outside of the physiological benefits of creatine supplementation, research on the cognitive benefits exist. (seancochran.com)
2018). Populations suffering from reduced cognitive function have been shown to benefit from creatine supplementation (Machek et al. (seancochran.com)
2018). Findings also indicate creatine supplementation is able to increase markers signifying optimal brain homeostasis (Machek et al. (seancochran.com)
Literature and research provide two basic supplementation protocols for the creatine monohydrate. (seancochran.com)

Novel insights into mitochondrial molecular targets of iron-induced neurodegeneration: Reversal by cannabidiol, Vanessa Kappel da Silva et al. (grecc.org)
Several rare forms of congenital muscular dystrophy are not discussed in this article because of the lack of precise molecular and/or genetic information. (medscape.com)

It catalyzes the reversible transfer of the γ-phosphate group from ATP to creatine to yield ADP and creatine phosphate. (smith.edu)
In the muscles, a fraction of the total creatine binds to phosphate - forming creatine phosphate. (drugbank.com)
Creatine gets phosphorylated(binds with inorganic phosphate), just like ADP and these are high-energy chemical bonds. (naturalbodyinc.com)
Creatine can also help create more ATP via transferring these phosphate molecules to ADP, ensuring you have plenty of energy for training hard. (naturalbodyinc.com)
The ammonia(in blue) binds to bicarbonate anions(in red) to form carbamoyl phosphate, which then binds to ornithine to form citrulline. (naturalbodyinc.com)

Together they form a unique fingerprint. (elsevier.com)

Approximately 95% of total body creatine is found within skeletal muscles, an additional 5% is in tissues of the brain, eyes, kidneys, and testes (Smith-Ryan & Antonio, 2013). (seancochran.com)

Creatine is excreted as creatinine in the urine. (drugbank.com)

Creatine monohydrate is a dietary supplement utilized by the athletic and general populations. (seancochran.com)
Most of the current literature on creatine monohydrate has investigated the benefits of this supplement from a physiological perspective, it has also shown to improve cognitive parameters (Machek & Bagley, 2018). (seancochran.com)
1999). Studies indicate the overall muscular augmentation to be 20-50% with 5 grams of 4-6 times per day of creatine monohydrate (Haff et al. (seancochran.com)

Creatine kinase (CK) is an essential phosphoryl transferase enzyme in cellular energy homeostasis. (smith.edu)

The enhancement of mitochondrial function may also occur, but beyond some alterations in signalling and the mitochondrial biogenesis most other properties of PQQ are unlikely to extend to humans. (pharmaceuticalintelligence.com)

This presents a diagnostic challenge: when is a rare mitochondrial disease responsible for a more 'common or garden' neurological presentation, and how often are neurologists missing them in routine clinical practice? (bmj.com)

Drosophila melanogaster, the fruit fly, expresses both forms of striated muscle and benefits greatly from powerful genetic tools. (hopkinsmedicine.org)
1 , 2 Different genetic causes of mitochondrial disease may lead to very similar phenotypes, but on the other hand, very different clinical presentations can arise from the same underlying genetic cause. (bmj.com)
Most patients do not show the 'full house' of features typical of the canonical mitochondrial clinical syndromes, and unfortunately the clinical and genetic spectrum evolves on a yearly basis. (bmj.com)

Increases in creatine kinase with atorvastatin treatment are not associated with decreases in muscular performance. (umassmed.edu)
Over 70% of the studies performed on creatine have produced statistically significant improvements with an average 5-15% increases in work performance for maximal effort muscle contractions, maximal power/strength performance, and single/repetitive sport performances (Smith-Ryan et al. (seancochran.com)
2013). Though some research is evident of creatine and carbohydrate loading in unison increases glycogen storages in comparison to carbohydrate loading only (Smith-Ryan et al. (seancochran.com)

Mitochondrial disease may present with a multitude of clinical features in different combinations. (bmj.com)
Epilepsy in mitochondrial disease is usually accompanied by additional clinical features or is part of an epileptic encephalopathy. (bmj.com)

This graph shows the total number of publications written about "Creatine Kinase" by people in this website by year, and whether "Creatine Kinase" was a major or minor topic of these publications. (umassmed.edu)
This overview of the major mitochondrial 'chameleons' and 'mimics' will provide a guide ( box 1 ). (bmj.com)
Glutathionylation is the major form of S-thiolation in eukaryotic cells. (biomedcentral.com)

Supplemental creatine may have an energy-generating action during anaerobic exercise and may also have neuroprotective and cardioprotective actions. (drugbank.com)

In addition, resveratrol upregulated key signaling pathways associated with cellular homeostasis, including adenosine receptors, nuclear factor erythroid-derived 2-like 2 (Nrf2), heme oxygenase 1 (HO-1), sirtuin 1 (SIRT1), proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α), and phosphoinositide 3-kinase (PI3K). (bvsalud.org)

Following its biosynthesis, creatine is transported to the skeletal muscle, heart, brain and other tissues. (drugbank.com)

Charcot-Marie-Tooth disease: in particular axonal forms, but also demyelinating and intermediate forms, have been described in mitochondrial disease. (bmj.com)
Thus, rehabilitation in its original form is to conduct all treatments and support to help all kidney disease patients smoothly achieve social rehabilitation instead of simply implementing exercise therapy. (biomedcentral.com)
As few randomized and controlled studies are … published, much of the experience of the treatment of mitochondrial disease is based upon anecdotal reports and small case series, which demonstrate effect of some of the measures available. (iospress.com)

Structure of the mitochondrial creatine kinase octamer: high-resolution shadowing and image averaging of single molecules and formation of linear filaments under specific staining conditions. (silverchair.com)
The combination of high-resolution tantalum/tungsten (Ta/W) shadowing at very low specimen temperature (-250 degrees C) under ultrahigh vacuum (less than 2 x 10(-9) mbar) with circular harmonic image averaging revealed details on the surface structure of mitochondrial creatine kinase (Mi-CK) molecules with a resolution less than 2.5 nm. (silverchair.com)
NAC has a chemical structure, formed by the sulfhydryl functional group (-SH) plus an acetyl group (-COCH 3 ) linked to an amino group (NH 2 ). (zenithnutrition.com)

Creatine is an important compound for brain bioenergetics (Machek et al. (seancochran.com)

7] Obtain a creatine kinase level in all cases of suspected GSD. (medscape.com)
However, when iNOS (-/-) mice were crossed with manganese superoxide dismutase (MnSOD)-overexpressing animals, mitochondrial injury was ameliorated to the level of the wild type. (uky.edu)

Creatine is a essential, non-proteinaceous amino acid derivative found in all animals. (drugbank.com)

Anatomy17

Organisms7

Diseases9

Chemicals and Drugs113

Analytical, Diagnostic and Therapeutic Techniques and Equipment21

Phenomena and Processes28

Anthropology, Education, Sociology and Social Phenomena1

Information Science3

Heart failure affects mitochondrial but not myofibrillar intrinsic properties of skeletal muscle. (1/49)

Changes in mRNA expression profile underlie phenotypic adaptations in creatine kinase-deficient muscles. (2/49)

Mitochondrial creatine kinase: properties and function. (3/49)

Multiple interference of anthracyclines with mitochondrial creatine kinases: preferential damage of the cardiac isoenzyme and its implications for drug cardiotoxicity. (4/49)

Expression of creatine kinase isoenzyme genes during postnatal development of rat brain cerebellum: evidence for transcriptional regulation. (5/49)

Mitochondrial creatine kinase is critically necessary for normal myocardial high-energy phosphate metabolism. (6/49)

Differential effects of peroxynitrite on human mitochondrial creatine kinase isoenzymes. Inactivation, octamer destabilization, and identification of involved residues. (7/49)

Protective effect of creatine against inhibition by methylglyoxal of mitochondrial respiration of cardiac cells. (8/49)

Creatine Kinase

Creatine Kinase, Mitochondrial Form

Creatine

Mitochondria

Isoenzymes

Creatine Kinase, MM Form

Cytosol

Mitochondria, Liver

Creatine Kinase, BB Form

Molecular Sequence Data

Amino Acid Sequence

Base Sequence

Creatine Kinase, MB Form

Saccharomyces cerevisiae

Phosphatidylinositol 3-Kinases

Clinical Enzyme Tests

Phosphocreatine

MAP Kinase Signaling System

Protein Kinases

Protein-Serine-Threonine Kinases

Arginine Kinase

Muscles

Protein Kinase Inhibitors

Calcium-Calmodulin-Dependent Protein Kinases

src-Family Kinases

L-Lactate Dehydrogenase

Myocardium

Protein Kinase C

Rhabdomyolysis

p38 Mitogen-Activated Protein Kinases

Muscle, Skeletal

Electrophoresis, Cellulose Acetate

Adenosine Triphosphate

Cyclic AMP-Dependent Protein Kinases

Kinetics

Mitogen-Activated Protein Kinase 1

Pyruvate Kinase

Adenylate Kinase

p21-Activated Kinases

Adenosine Diphosphate

Mitogen-Activated Protein Kinase Kinases

JNK Mitogen-Activated Protein Kinases

Guanidinoacetate N-Methyltransferase

Mitogen-Activated Protein Kinase 3

Muscular Diseases

Magnetic Resonance Spectroscopy

Amidinotransferases

Protein-Tyrosine Kinases

Rabbits

Myoglobin

Time Factors

CDC2 Protein Kinase

Cyclin-Dependent Kinases

Phosphorylation

MAP Kinase Kinase Kinases

Myocardial Infarction

eIF-2 Kinase

Casein Kinase II

Casein Kinases

Mitochondria, Heart

Ribosomal Protein S6 Kinases

MAP Kinase Kinase 1

Myositis

Energy Metabolism

Aspartate Aminotransferases

Receptor Protein-Tyrosine Kinases

Thymidine Kinase

Electrophoresis, Agar Gel

Protein Binding

Extracellular Signal-Regulated MAP Kinases

MAP Kinase Kinase 4

Brain

Troponin T

Troponin I

Signal Transduction

1-Phosphatidylinositol 4-Kinase

Phosphotransferases (Alcohol Group Acceptor)

Mitogen-Activated Protein Kinases

CDC2-CDC28 Kinases

Enzyme Activation