3-Hydroxyacyl CoA Dehydrogenases: Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in the presence of NAD. They are key enzymes in the oxidation of fatty acids and in mitochondrial fatty acid synthesis.Acyl-CoA Dehydrogenases: Enzymes that catalyze the first step in the beta-oxidation of FATTY ACIDS.Acyl-CoA Dehydrogenase: A flavoprotein oxidoreductase that has specificity for medium-chain fatty acids. It forms a complex with ELECTRON TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.Lipid A: Lipid A is the biologically active component of lipopolysaccharides. It shows strong endotoxic activity and exhibits immunogenic properties.Acyltransferases: Enzymes from the transferase class that catalyze the transfer of acyl groups from donor to acceptor, forming either esters or amides. (From Enzyme Nomenclature 1992) EC 2.3.Fatty Acids: Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)Dictionaries, MedicalEnoyl-CoA Hydratase: An enzyme that catalyzes reversibly the hydration of unsaturated fatty acyl-CoA to yield beta-hydroxyacyl-CoA. It plays a role in the oxidation of fatty acids and in mitochondrial fatty acid synthesis, has broad specificity, and is most active with crotonyl-CoA. EC 4.2.1.17.Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase: An NAD-dependent 3-hydroxyacyl CoA dehydrogenase that has specificity for acyl chains containing 8 and 10 carbons.Mitochondrial Trifunctional Protein: A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. It contains enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, and acetyl-CoA C-acyltransferase activities and plays an important role in the metabolism of long chain FATTY ACIDS.Peroxisomal Bifunctional Enzyme: A monomeric protein found in liver peroxisomes that contains two enzymatically active domains; an enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase domain, and an (S)-3-hydroxyacyl-CoA dehydrogenase domain. The enzyme is stereospecific with regards to how cis and trans double bonds are metabolized. It is complemented by PEROXISOMAL MULTIFUNCTIONAL PROTEIN-2, which has the opposite stereospecificity.Peroxisomal Multifunctional Protein-2: A dimeric protein found in liver peroxisomes that plays an important role in FATTY ACID metabolism and steroid metabolism. The dimer is formed by cleavage of a single protein precursor and contains an enoyl-CoA hydratase-2 domain and a second domain that displays (S)-3-hydroxyacyl-CoA dehydrogenase and 17-beta-estradiol dehydrogenase activities. The enzyme is stereospecific with regards to arrangement of the substrate double bonds and position of the 3-hydroxy group of the reaction intermediate. It is complemented by PEROXISOMAL BIFUNCTIONAL ENZYME, which has the opposite reaction stereospecificity.Metabolome: The dynamic collection of metabolites which represent a cell's or organism's net metabolic response to current conditions.Metabolomics: The systematic identification and quantitation of all the metabolic products of a cell, tissue, organ, or organism under varying conditions. The METABOLOME of a cell or organism is a dynamic collection of metabolites which represent its net response to current conditions.Visual Acuity: Clarity or sharpness of OCULAR VISION or the ability of the eye to see fine details. Visual acuity depends on the functions of RETINA, neuronal transmission, and the interpretative ability of the brain. Normal visual acuity is expressed as 20/20 indicating that one can see at 20 feet what should normally be seen at that distance. Visual acuity can also be influenced by brightness, color, and contrast.Central Serous Chorioretinopathy: A visual impairment characterized by the accumulation of fluid under the retina through a defect in the retinal pigment epithelium.Lipid Metabolism, Inborn Errors: Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.Glucosephosphate Dehydrogenase Deficiency: A disease-producing enzyme deficiency subject to many variants, some of which cause a deficiency of GLUCOSE-6-PHOSPHATE DEHYDROGENASE activity in erythrocytes, leading to hemolytic anemia.Nigella sativa: A plant genus of the family RANUNCULACEAE that contains alpha-hederin, a triterpene saponin in the seeds, and is the source of black seed oil.Antisense Elements (Genetics): Nucleic acids which hybridize to complementary sequences in other target nucleic acids causing the function of the latter to be affected.Peptide Nucleic Acids: DNA analogs containing neutral amide backbone linkages composed of aminoethyl glycine units instead of the usual phosphodiester linkage of deoxyribose groups. Peptide nucleic acids have high biological stability and higher affinity for complementary DNA or RNA sequences than analogous DNA oligomers.Theonella: A genus of shallow-cupped SPONGES with a broad base in the family Theonellidae. They are characterized by ectosomal spicules dominated by phyllotriaenes.Peptides: Members of the class of compounds composed of AMINO ACIDS joined together by peptide bonds between adjacent amino acids into linear, branched or cyclical structures. OLIGOPEPTIDES are composed of approximately 2-12 amino acids. Polypeptides are composed of approximately 13 or more amino acids. PROTEINS are linear polypeptides that are normally synthesized on RIBOSOMES.Amyloid beta-Peptides: Peptides generated from AMYLOID BETA-PEPTIDES PRECURSOR. An amyloid fibrillar form of these peptides is the major component of amyloid plaques found in individuals with Alzheimer's disease and in aged individuals with trisomy 21 (DOWN SYNDROME). The peptide is found predominantly in the nervous system, but there have been reports of its presence in non-neural tissue.Alzheimer Disease: A degenerative disease of the BRAIN characterized by the insidious onset of DEMENTIA. Impairment of MEMORY, judgment, attention span, and problem solving skills are followed by severe APRAXIAS and a global loss of cognitive abilities. The condition primarily occurs after age 60, and is marked pathologically by severe cortical atrophy and the triad of SENILE PLAQUES; NEUROFIBRILLARY TANGLES; and NEUROPIL THREADS. (From Adams et al., Principles of Neurology, 6th ed, pp1049-57)Peptide Fragments: Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.Fatigue: The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli.Databases, Protein: Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.Oxidation-Reduction: A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).Sequence Analysis, Protein: A process that includes the determination of AMINO ACID SEQUENCE of a protein (or peptide, oligopeptide or peptide fragment) and the information analysis of the sequence.Blood Platelets: Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation.Diagnostic Errors: Incorrect diagnoses after clinical examination or technical diagnostic procedures.Platelet Count: The number of PLATELETS per unit volume in a sample of venous BLOOD.Cough: A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation. It is a protective response that serves to clear the trachea, bronchi, and/or lungs of irritants and secretions, or to prevent aspiration of foreign materials into the lungs.Megakaryocytes: Very large BONE MARROW CELLS which release mature BLOOD PLATELETS.Thrombopoiesis: The process of generating thrombocytes (BLOOD PLATELETS) from the pluripotent HEMATOPOIETIC STEM CELLS in the BONE MARROW via the MEGAKARYOCYTES. The humoral factor with thrombopoiesis-stimulating activity is designated THROMBOPOIETIN.Thrombopoietin: A humoral factor that stimulates the production of thrombocytes (BLOOD PLATELETS). Thrombopoietin stimulates the proliferation of bone marrow MEGAKARYOCYTES and their release of blood platelets. The process is called THROMBOPOIESIS.Euphoria: An exaggerated feeling of physical and emotional well-being not consonant with apparent stimuli or events; usually of psychologic origin, but also seen in organic brain disease and toxic states.Dysarthria: Disorders of speech articulation caused by imperfect coordination of pharynx, larynx, tongue, or face muscles. This may result from CRANIAL NERVE DISEASES; NEUROMUSCULAR DISEASES; CEREBELLAR DISEASES; BASAL GANGLIA DISEASES; BRAIN STEM diseases; or diseases of the corticobulbar tracts (see PYRAMIDAL TRACTS). The cortical language centers are intact in this condition. (From Adams et al., Principles of Neurology, 6th ed, p489)Paresthesia: Subjective cutaneous sensations (e.g., cold, warmth, tingling, pressure, etc.) that are experienced spontaneously in the absence of stimulation.Nurse Clinicians: Registered nurses who hold Master's degrees in nursing with an emphasis in clinical nursing and who function independently in coordinating plans for patient care.Gait Disorders, Neurologic: Gait abnormalities that are a manifestation of nervous system dysfunction. These conditions may be caused by a wide variety of disorders which affect motor control, sensory feedback, and muscle strength including: CENTRAL NERVOUS SYSTEM DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; NEUROMUSCULAR DISEASES; or MUSCULAR DISEASES.Nursing Evaluation Research: Research carried out by nurses that uses interviews, data collection, observation, surveys, etc., to evaluate nursing, health, clinical, and nursing education programs and curricula, and which also demonstrates the value of such evaluation.Gait: Manner or style of walking.Chorea Gravidarum: A rare movement disorder developed during PREGNANCY, characterized by involuntary jerky motion (CHOREA) and inability to maintain stable position of body parts (ATHETOSIS). RHEUMATIC FEVER and collagen vascular disorders are frequently associated with this disease. Chorea may vary from mild to severe and occurs in approximately 1 per 2,000 to 3,000 pregnancies. (From Md Med J 1997 Sep;46(8):436-9)Search Engine: Software used to locate data or information stored in machine-readable form locally or at a distance such as an INTERNET site.Databases, Genetic: Databases devoted to knowledge about specific genes and gene products.Dodecenoyl-CoA Isomerase: A carbon-carbon double bond isomerase that catalyzes the movement double bond from C3 to C2 of an unsaturated acyl-CoA. The enzyme plays a key role in allowing acyl-CoA substrates to re-enter the beta-oxidation pathway.Carbon-Carbon Double Bond Isomerases: Enzymes that catalyze the shifting of a carbon-carbon double bond from one position to another within the same molecule. EC 5.3.3.Isomerases: A class of enzymes that catalyze geometric or structural changes within a molecule to form a single product. The reactions do not involve a net change in the concentrations of compounds other than the substrate and the product.(from Dorland, 28th ed) EC 5.alpha-Linolenic Acid: A fatty acid that is found in plants and involved in the formation of prostaglandins.Plant Oils: Oils derived from plants or plant products.Fatty Liver: Lipid infiltration of the hepatic parenchymal cells resulting in a yellow-colored liver. The abnormal lipid accumulation is usually in the form of TRIGLYCERIDES, either as a single large droplet or multiple small droplets. Fatty liver is caused by an imbalance in the metabolism of FATTY ACIDS.DiglyceridesLiver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.Lipid Metabolism: Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS.Diacylglycerol Kinase: An enzyme of the transferase class that uses ATP to catalyze the phosphorylation of diacylglycerol to a phosphatidate. EC 2.7.1.107.

Metabolic adaptation of two pig muscles to cold rearing conditions. (1/1)

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isovaleryl-CoA dehydrogenase Ja 1.3.99.13 long-chain-acyl-CoA dehydrogenase Ja ... L-iditol 2-dehydrogenase Ja 1.1.1.15 D-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. D-sorbose + NADH + H+ D-iditol 2- ... D-arabitol 4-dehydrogenase 1.1.1.12 L-arabitol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-xylulose + NADH + H+ L-arabitol ... L-arabitol 2-dehydrogenase 1.1.1.14 L-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-sorbose + NADH + H+ ...
SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... PC Pyruvate dehydrogenase deficiency; 312170; PDHA1 Pyruvate dehydrogenase E2 deficiency; 245348; DLAT Pyruvate dehydrogenase ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... TMPRSS6 Isobutyryl-CoA dehydrogenase deficiency; 611283; ACAD8 Isovaleric acidemia; 243500; IVD IVIC syndrome; 147750; SALL4 ...
"hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit". Cardiac ... Trifunctional enzyme subunit alpha, mitochondrial also known as hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA ... Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit". Zong NC, Li ... The enzyme converts medium- and long-chain 2-enoyl-CoA compounds into the following 3-ketoacyl-CoA when NAD is solely present, ...
HSD17B4 "EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. 6 September 2017. ...
Wanders RJ, Vreken P, den Boer ME, Wijburg FA, van Gennip AH, IJlst L (1999). "Disorders of mitochondrial fatty acyl-CoA beta- ... Deficiency of LCHAD (3-hydroxyacyl-CoA dehydrogenase) leads to an accumulation of medium and long chain fatty acids. When this ... It is thought to be caused by a disordered metabolism of fatty acids by mitochondria in the mother, caused by long-chain 3- ... hydroxyacyl-coenzyme A dehydrogenase deficiency. The condition was previously thought to be universally fatal, but aggressive ...
1998). "Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in ... acyl-CoA oxidase (see, e.g., ACOX1, MIM 609751); the 'D-bifunctional enzyme,' with enoyl-CoA hydratase and D-3-hydroxyacyl-CoA ... 1999). "17Beta-hydroxysteroid dehydrogenases in human bone cells". J. Bone Miner. Res. 13 (10): 1539-46. doi:10.1359/jbmr. ... 1999). "17Beta-hydroxysteroid dehydrogenase type 1, 2, 3, and 4 expression and enzyme activity in human anterior pituitary ...
Lactate dehydrogenase is a marker of hemolysis and is elevated (>600 U/l). Proteinuria is present but can be mild. In one 1995 ... HELLP is characterized by hemolysis on peripheral blood smear with serum lactate dehydrogenase >600 IU/l; serum aspartate ... 60 (3): 829-36, 839. PMID 10498110. Gillingham M, Van Calcar S, Ney D, Wolff J, Harding C. Dietary management of long-chain 3- ... hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey. Journal of inherited metabolic disease. 1999;22(2 ...
"Binding of amyloid beta-peptide to mitochondrial hydroxyacyl-CoA dehydrogenase (ERAB): regulation of an SDR enzyme activity ... 17beta-hydroxysteroid dehydrogenase 10 is a member of the short-chain dehydrogenase/reductase superfamily. This homotetrameric ... He XY, Yang YZ, Schulz H, Yang SY (Jan 2000). "Intrinsic alcohol dehydrogenase and hydroxysteroid dehydrogenase activities of ... "Intrinsic alcohol dehydrogenase and hydroxysteroid dehydrogenase activities of human mitochondrial short-chain L-3-hydroxyacyl- ...
... , ECHB, MSTP029, MTPB, TP-BETA, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional ... hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta. External IDs. OMIM: 143450 MGI: 2136381 ... acetyl-CoA C-acyltransferase activity. • long-chain-enoyl-CoA hydratase activity. Cellular component. • membrane. • ... "Entrez Gene: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional ...
Dehydrogenation by acyl-CoA dehydrogenase, yielding 1 FADH2 Hydration by enoyl-CoA hydratase Dehydrogenation by 3-hydroxyacyl- ... Acetyl-CoA is formed into malonyl-CoA by acetyl-CoA carboxylase, at which point malonyl-CoA is destined to feed into the fatty ... CoA dehydrogenase, yielding 1 NADH + H+ Cleavage by thiolase, yielding 1 acetyl-CoA and a fatty acid that has now been ... acetyl-CoA and 1 molecule of propionyl-CoA per molecule of fatty acid. Each beta oxidative cut of the acyl-CoA molecule yields ...
... hypoglycaemia Levomepromazine Liver cancer Liver glycogen synthase deficiency Long-chain hydroxyacyl-CoA dehydrogenase ... Trimethoprim Triple A syndrome Tumors Tyrosinaemia type 1 Urea cycle disorder Uremia Very-long-chain acyl-CoA dehydrogenase ... Reye syndrome Ritonavir Saquinavir Sepsis Septic shock Severe hepatitis Sheehan syndrome Short-chain acyl-CoA dehydrogenase ... deficiency Maple syrup urine disease Mcquarrie type infantile idiopathic hypoglycemia Medium chain acyl-CoA dehydrogenase ...
"OMIM Entry - * 600890 - HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, ALPHA SUBUNIT; HADHA". omim. ... "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Homo ... "Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ...
... glutaryl-CoA dehydrogenase EC 1.3.8.7: medium-chain acyl-CoA dehydrogenase EC 1.3.8.8: long-chain acyl-CoA dehydrogenase EC 1.3 ... glutaryl-CoA dehydrogenase EC 1.3.99.8: 2-furoyl-CoA dehydrogenase EC 1.3.99.9: now *EC 1.21.99.1 EC 1.3.99.10: isovaleryl-CoA ... succinate dehydrogenase EC 1.3.99.2: butyryl-CoA dehydrogenase EC 1.3.99.3: acyl-CoA dehydrogenase EC 1.3.99.4: 3-oxosteroid 1- ... benzylsuccinyl-CoA dehydrogenase EC 1.3.8.4: isovaleryl-CoA dehydrogenase EC 1.3.8.5: 2-methyl-branched-chain-enoyl-CoA ...
... namely acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and thiolase. The cycle produces a new ... It regulates through the ratio of acetyl-CoA versus CoA. Increased concentration of acetyl-CoA activates PDK. Acetyl-CoA is ... Acetyl-CoA can be carboxylated in the cytosol by acetyl-CoA carboxylase, giving rise to malonyl-CoA, a substrate required for ... "Regulation of pyruvate dehydrogenase kinase and phosphatase by acetyl-CoA/CoA and NADH/NAD ratios". Biochemical and Biophysical ...
... hydroxyacyl coenzyme-A dehydrogenase, NADP+-linked acetoacetyl CoA reductase, NADPH:acetoacetyl-CoA reductase, D(−)-beta- ... hydroxybutyryl CoA-NADP+ oxidoreductase, short chain beta-ketoacetyl(acetoacetyl)-CoA reductase, beta-ketoacyl-CoA reductase, D ... In enzymology, an acetoacetyl-CoA reductase (EC 1.1.1.36) is an enzyme that catalyzes the chemical reaction (R)-3-hydroxyacyl- ... CoA + NADP+ ⇌ {\displaystyle \rightleftharpoons } 3-oxoacyl-CoA + NADPH + H+ Thus, the two substrates of this enzyme are (R)-3- ...
The dehydrogenase activity of enoyl-CoA occurs in the carboxyl-terminal. Upon further investigation of the CoA binding site on ... delta2-Enoyl-CoA Isomerase and Peroxisomal Multifunctional delta3,delta2- Enoyl-CoA Isomerase, 2-Enoyl-CoA Hydratase, 3- ... Hydroxyacyl-CoA Dehydrogenase Enzyme in Rat Liver" (PDF). Journal of Biological Chemistry. 266 (17): 10750-10753. PMID 2040594 ... Enoyl-CoA-(∆) isomerase, also known as dodecenoyl-CoA-(∆) isomerase, 3,2-trans-enoyl-CoA isomerase, ∆3(cis),∆2(trans)-enoyl-CoA ...
L-hydroxyacyl-CoA dehydrogenase 3beta-hydroxyacyl coenzyme A dehydrogenase beta-hydroxy acid dehydrogenase beta-hydroxyacyl CoA ... dehydrogenase beta-hydroxyacyl dehydrogenase beta-hydroxyacyl-coenzyme A synthetase beta-hydroxyacylcoenzyme A dehydrogenase ... beta-Hydroxyacyl coenzyme A dehydrogenase". J. Biol. Chem. 207 (2): 631-8. PMID 13163047. Molecular and Cellular Biology portal ... Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme ...
... such that HIV-1 Vpr regulates mitochondrial respiration and enhances the activity of hydroxyacyl-CoA dehydrogenase (HADH) ... Hydroxyacyl-Coenzyme A dehydrogenase also known as HADH is an enzyme which in humans is encoded by the HADH gene. The HADH gene ... "Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB). Molven A, Matre ... "A preliminary analysis of the segregation of human hydroxyacyl coenzyme A dehydrogenase in human-mouse somatic cell hybrids". ...
... (EC 1.3.8.1, butyryl-CoA dehydrogenase, butanoyl-CoA dehydrogenase, butyryl dehydrogenase, ... butyryl coenzyme A dehydrogenase, short-chain acyl CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, 3-hydroxyacyl ... Short-chain acyl-CoA dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH) Molecular and ... Thorpe, C.; Kim, J.J. (1995). "Structure and mechanism of action of the acyl-CoA dehydrogenases". FASEB J. 9 (9): 718-725. PMID ...
People with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency have inadequate levels of an enzyme required for a step that ... Problems related to 3-hydroxyacyl-coenzyme A dehydrogenase deficiency can be triggered by periods of fasting or by illnesses ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (HADH deficiency) is a rare condition that prevents the body from converting ... Individuals with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency are also at risk for complications such as seizures, life- ...
CoA) dehydrogenase, which is part of a protein complex known as mitochondrial trifunctional protein. Long-chain fatty acids ... Medium chain acyl dehydrogenase deficiency Reference, Genetics Home. "LCHAD deficiency". Genetics Home Reference. Retrieved ... Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, often shortened to LCHAD deficiency, is a rare autosomal ... Mutations in the HADHA gene lead to inadequate levels of an enzyme called long-chain 3-hydroxyacyl-coenzyme A ( ...
Relationship between the different dehydrogenases and evidence that fatty acids and the C27 bile acids di- and tri- ... An inducible fatty acyl-CoA oxidase, a noninducible fatty acyl-CoA oxidase, and a noninducible trihydroxycoprostanoyl-CoA ... THC-CoA oxidase, THCA-CoA oxidase, 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA oxidase, 3alpha,7alpha,12alpha- ... Schepers L, Van Veldhoven PP, Casteels M, Eyssen HJ, Mannaerts GP (1990). "Presence of three acyl-CoA oxidases in rat liver ...
4 Dienoyl-CoA reductase deficiency - 2,4 Dienoyl-CoA reductase 3-hydroxy-3-methylglutaryl-CoA lyase deficiency - 3-hydroxy-3- ... Medium-chain acyl-coenzyme A dehydrogenase Short-chain acyl-coenzyme A dehydrogenase deficiency (SCAD) - Short-chain acyl- ... methylglutaryl-CoA lyase Malonyl-CoA decarboxylase deficiency - Malonyl-CoA decarboxylase Primary carnitine deficiency - ... Some of the more common fatty acid metabolism disorders are: Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD ...
Acyl CoA dehydrogenase, Enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase. Since lipids (fats) ... The resulting Acyl-CoA activates the process of beta oxidation to further break down into Acetyl-CoA (which is used in the ... Fatty acid metabolism begins in the cytoplasm of epithelial cells as Acyl-CoA synthetase and hydrolysis of ATP cleaves a ... King, Michael W. Fatty Acid, Omega-3 and Omega-6 Fatty Acid, Triglyceride, and Phospholipid Synthesis and Metabolism. The ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... Thus, the two substrates of this enzyme are xylitol and NADP+, whereas its 3 products are L-xylulose, NADPH, and H+. ... Over-expression and ectopic expression of the protein may be associated with prostate adenocarcinoma.[3] ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... In enzymology, a homoisocitrate dehydrogenase (EC 1.1.1.87) is an enzyme that catalyzes the chemical reaction ... Rowley B, Tucci AF (1970). "Homoisocitric dehydrogenase from yeast". Arch. Biochem. Biophys. 141 (2): 499-, 510. doi:10.1016/ ... homoisocitric dehydrogenase, (−)-1-hydroxy-1,2,4-butanetricarboxylate:NAD+ oxidoreductase, (decarboxylating), 3-carboxy-2- ...
Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency. Biochem Biophys Res Commun. 2008 Mar 28;368(1):6-11. ... Short-chain hydroxyacyl-coenzyme A dehydrogenase deficiency presenting as unexpected infant death: A family study. J Pediatr. ... Problems related to 3-hydroxyacyl-CoA dehydrogenase deficiency can be triggered by periods of fasting or by illnesses such as ... Mutations in the HADH gene cause 3-hydroxyacyl-CoA dehydrogenase deficiency. The HADH gene provides instructions for making an ...
L-hydroxyacyl-CoA dehydrogenase 3beta-hydroxyacyl coenzyme A dehydrogenase beta-hydroxy acid dehydrogenase beta-hydroxyacyl CoA ... dehydrogenase beta-hydroxyacyl dehydrogenase beta-hydroxyacyl-coenzyme A synthetase beta-hydroxyacylcoenzyme A dehydrogenase ... beta-Hydroxyacyl coenzyme A dehydrogenase". J. Biol. Chem. 207 (2): 631-8. PMID 13163047. Molecular and Cellular Biology portal ... Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme ...
Antibodies for proteins involved in 3-hydroxyacyl-CoA dehydrogenase activity pathways, according to their Panther/Gene Ontology ... Antibodies for proteins involved in 3-hydroxyacyl-CoA dehydrogenase activity pathways; according to their Panther/Gene Ontology ... Host server : magellan-srch-3-prod-blue:8080/10.253.228.147:8080. git-commit: b6907141bf9ff2f81bd54e0bfa97b1a010a74181 git-url ...
Recognition of Structurally Diverse Substrates by Type II 3-Hydroxyacyl-Coa Dehydrogenase (Hadh II) Amyloid-Beta Binding ... 3] Andreeva A., Howorth D., Chandonia J.-M., Brenner S.E., Hubbard T.J.P., Chothia C., Murzin A.G. (2008).. Data growth and its ... Rat brain 3-hydroxyacyl-CoA dehydrogenase binary complex with NADH and 3-keto butyrate. ... If available, the SCOP 1.75 domain assignment [3] is used. Otherwise algorithmic domain assignments are computed using the ...
Hydroxyacyl-CoA dehydrogenase (EC1.1.1.35) from mitochondria at the resolution 2.0 A, Northeast Structural Genomics Consortium ... Hydroxyacyl-CoA dehydrogenase (EC1.1.1.35) from mitochondria at the resolution 2.0 A, Northeast Structural Genomics Consortium ... Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial. A, B. 324. Homo sapiens. Mutation(s): 0 Gene Names: HADH (HAD, HADHSC, ... GLYCERIN; PROPANE-1,2,3-TRIOL. C3 H8 O3. PEDCQBHIVMGVHV-UHFFFAOYSA-N. ...
CoA) Dehydrogenase (LCHAD) Associated Neuropathy. The safety and scientific validity of this study is the responsibility of the ... Long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD). Mitochondrial trifunctional protein (TFP). LCHAD/TFP deficiency with ... Vitamin E Treatment for Long-Chain 3-Hydroxyacyl Coenzyme A ( ...
What is 3-hydroxyacyl-CoA dehydrogenase? Meaning of 3-hydroxyacyl-CoA dehydrogenase medical term. What does 3-hydroxyacyl-CoA ... Looking for online definition of 3-hydroxyacyl-CoA dehydrogenase in the Medical Dictionary? 3-hydroxyacyl-CoA dehydrogenase ... 3-hy·drox·y·ac·yl-CoA de·hy·dro·gen·ase. (hī-droksē-asil dēhī-drōjen-ā), β-Hydroxyacyl dehydrogenase; an enzyme catalyzing ... Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: high frequency of the G1528C mutation with no apparent correlation with ...
2-Methylacetoacetyl-CoA + NADH. details 2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Ion. ... Submicroscopic duplications of the hydroxysteroid dehydrogenase HSD17B10 and the E3 ubiquitin ligase HUWE1 are associated with ... 2004 Sep 17;342(3):943-52. [PubMed:15342248 ] *Lustbader JW, Cirilli M, Lin C, Xu HW, Takuma K, Wang N, Caspersen C, Chen X, ... S)-3-hydroxyacyl-CoA + NAD → 3-oxoacyl-CoA + NADH. details 2-Methyl-3-hydroxybutyryl-CoA + NAD → ...
86, nr 3, 329-337 s. Nyckelord [en] Chorioretinal atrophy, electroetinography, LCHAD deficiency, myopia Nationell ämneskategori ... 86, nr 3, 329-337 s.Artikel i tidskrift (Refereegranskat) Published Abstract [en] PURPOSE ... Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a cross-sectional study with ... To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA ...
Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)Download version for offline viewing or printing. [463.65kB] ... Babies with the diseases Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) or Trifunctional Protein Deficiency (TFP ...
... and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency are long-chain fatty acid oxidation disorders with ... For very-long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the most common long-chain fatty oxidation defect, it has been ... Ficicioglu C, Coughlin CR 2nd, Bennett MJ, Yudkoff M. Very long-chain acyl-CoA dehydrogenase deficiency in a patient with ... A near-miss: very long chain acyl-CoA dehydrogenase deficiency with normal primary markers in the initial well-timed newborn ...
The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase ( ... 2002). Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients. Pediatrics. ... LCHAD (Long-Chain 3-Hydroxyacyl CoA Dehydrogenase) deficiency. LCHAD (long-chain 3-hydroxyacyl CoA dehydrogenase) deficiency is ... Chronically 3-hydroxylated dicarboxylic acids and nonhydroxylated dicarboxylic acids will be positive. Plasma carnitine may be ...
... glutamate dehydrogenase; immune response; recombinant proteins; surface proteins; vaccine development. Abstract:. ... ... and glutamate dehydrogenase (gdhA)) against Flavobacterium psychrophilum were investigated in ayu (Plecoglossus altivelis). ... 3-hydroxyacyl-CoA dehydrogenase; Escherichia coli; Flavobacterium psychrophilum; H+/K+-exchanging ATPase; H-transporting ATP ... Protective efficacies of three antigenic proteins (3-hydroxyacyl-CoA dehydrogenase (HCD), ATP synthase beta subunit (atpD), ...
... of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for 3-alpha ... 3-alpha hydroxyacyl-CoA dehydrogenase deficiency Title Other Names:. 3-hydroxylacyl-CoA dehydrogenase deficiency; Medium and ... PubMed is a searchable database of medical literature and lists journal articles that discuss 3-alpha hydroxyacyl-CoA ... dehydrogenase deficiency. Click on the link to view a sample search on this topic. ...
Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase, including: function, proteins, disorders, pathways, orthologs, and ... Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase 2 3 5 * Enoyl-Coenzyme A, Hydratase/3-Hydroxyacyl Coenzyme A ... EHHADH (Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase) is a Protein Coding gene. Diseases associated with EHHADH ... The GeneCards human gene database index: 2 3 5 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z ...
Compare Anti-Alcohol Dehydrogenase 2 Antibody Products from leading suppliers on Biocompare. View specifications, prices, ... Rabbit anti-human zinc binding alcohol dehydrogenase domain containing 2 polyclonal Antibody ...
isovaleryl-CoA dehydrogenase Ja 1.3.99.13 long-chain-acyl-CoA dehydrogenase Ja ... L-iditol 2-dehydrogenase Ja 1.1.1.15 D-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. D-sorbose + NADH + H+ D-iditol 2- ... D-arabitol 4-dehydrogenase 1.1.1.12 L-arabitol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-xylulose + NADH + H+ L-arabitol ... L-arabitol 2-dehydrogenase 1.1.1.14 L-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-sorbose + NADH + H+ ...
The most important finding of this study was the detection of the lipid-metabolizing enzyme, 3-hydroxyacyl-CoA dehydrogenase ( ... From: Identification of leptospiral 3-hydroxyacyl-CoA dehydrogenase released in the urine of infected hamsters ...
... medium-chain acyl-CoA dehydrogenase deficiency (Table) (3.6). Newborn screening can help prevent death or disability, if ... The five most commonly diagnosed conditions in the United States are 1) hearing loss, 2) primary congenital hypothyroidism, 3) ... 3). Additional conditions for screening continue to be identified and nominated for inclusion in the panel. ... 3). Through early identification, newborn screening provides an opportunity for treatment and significant reductions in ...
We describe two children with deficiency of short-chain L-3-hydroxyacyl-CoA dehydrogenase, a new disorder of the mitochondrial ... Mitochondrial Short-Chain L-3-hydroxyacyl-coenzyme A Dehydrogenase Deficiency: A New Defect of Fatty Acid Oxidation Pediatr Res ... We describe two children with deficiency of short-chain L-3-hydroxyacyl-CoA dehydrogenase, a new disorder of the mitochondrial ...
Fraser H1, Geppert J2, Johnson R3, Johnson S4, Connock M2, Clarke A2, Taylor-Phillips S2, Stinton C2. ... 3. Faculty of Health and Life Sciences, Coventry University, Coventry, CV1 5RW, UK.. 4. Warwick Library, University of Warwick ... 3. Forest plot showing mortality and incidence of cardiac and liver problems across symptomatically and asymptomatically ... For follow up analysis 3 we found a significant difference for only one comparison, in the incidence of cardiomyopathy between ...
Catalase and the endoplasmic reticulum A beta binding dehydrogenase (ERAB) are both inhibited by characterized fragments of the ... Catalase and the endoplasmic reticulum A beta binding dehydrogenase (ERAB) are both inhibited by characterized fragments of the ...
GO:0004300 enoyl-CoA hydratase activity Cellular Component. GO:0016507 mitochondrial fatty acid beta-oxidation multienzyme ... Enoyl-CoA hydratase/isomerase (IPR001753) *Fatty acid oxidation complex, alpha subunit, mitochondrial (IPR012803) ... Subunit activities include: enoyl-CoA hydratase (EC:4.2.1.17) and 3-hydroxyacyl-CoA dehydrogenase (EC:1.1.1.35). Some ... The beta subunit has acetyl-CoA C-acyltransferase (EC:2.3.1.16) activity. ...
Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Long chain/very long chain acyl CoA dehydrogenase deficiency, ... Long chain/very long chain acyl CoA dehydrogenase deficiency, ...
... hydroxyacyl-CoA dehydrogenase fragment of rat peroxisomal MFE-2 (PDB code 1GZ6) and molecule A of the (2E)-enoyl-CoA hydratase ... hydroxyacyl-CoA dehydrogenase domain, a C-terminal (2E)-enoyl-CoA hydratase 2 domain and a short loop which connects the two ... Hydroxyacyl-CoA dehydrogenase domain of Dm. MFE-2. The (3R)-hydroxyacyl-CoA dehydrogenase domain of DmMFE-2 consists of amino ... Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional ...
  • The beta subunit has acetyl-CoA C-acyltransferase ( EC:2.3.1.16 ) activity. (ebi.ac.uk)
  • Enzymes catalyzing reactions in phosphagenic [creatine kinase (CK)] and glycolytic [hexokinase (HK), phosphofructokinase (PFK), and lactate dehydrogenase (LDH)] metabolic pathways had significantly higher activities (about 30-40%) in the Black African group than in the Caucasian group (P less than 0.01). (physicsforums.com)
  • It was the aim of this study to elucidate the organization of its CoA-derived pathways and to use the genomic information of X. dendrorhous for a phylogenomic investigation of the Basidiomycota. (biomedcentral.com)
  • A detailed characterization of the CoA-related pathways was done and all genes for fatty acid, sterol and carotenoid synthesis have been assigned. (biomedcentral.com)
  • Recombinant fragment containing a sequence corresponding to a region within amino acids 18-231 of Human hydroxysteroid (17-beta) dehydrogenase 4 (AAH03098). (abcam.com)
  • Overexpression of 17β-hydroxysteroid dehydrogenase type 10 increases pheochromocytoma cell growth and resistance to cell death. (nih.gov)
  • 17β-hydroxysteroid dehydrogenase type 10 (HSD10) has been shown to play a protective role in cells undergoing stress. (nih.gov)
  • Furthermore, to investigate the catalytic residues of CacHBD, the enzymatic activities of the wild type and of three single-amino-acid mutants were analyzed, in which the Ser, His and Asn residues that are conserved in the HBDs from C. acetobutylicum , C. butyricum and Ralstonia eutropha , as well as in the L-3-hydroxyacyl-CoA dehydrogenases from Homo sapiens and Escherichia coli , were substituted by alanines. (proteopedia.org)
  • Our results reveal that rapamycin inhibits the phosphorylation of TORC1 and its downstream effectors (S6K1, S6 and 4E-BP1), without affecting Akt and the Akt substrates Forkhead-box Class O1 (FoxO1) and glycogen synthase kinase 3α/β (GSK 3α/β). (biologists.org)
  • The ability of insulin to phosphorylate Akt and activate glycogen synthase (GS) was reduced with normal GS site 3 but abnormal GS site 2+2a phosphorylation after bed rest. (diabetesjournals.org)
  • These are organic compounds containing a 3-hydroxyl acylated coenzyme A derivative. (hmdb.ca)
  • A molecule of coenzyme A carrying an acetyl group is also referred to as acetyl-CoA. (hmdb.ca)
  • These are organic compounds containing a coenzyme A derivative, which is 3-oxo acylated long aliphatic chain of 13 to 21 carbon atoms. (hmdb.ca)
  • Second, their enormous genomes, often in excess of 10 Mbp, consist of single circular chromosomes filled with seemingly redundant genes ( 3 , 4 ). (asm.org)
  • A simple, straightforward assay for long-chain 3-hydroxyacyl-CoA dehydrogenase based on the use of 4ethylmaleimide: potential for pre and postnatal diagnosis. (thefreedictionary.com)
  • belongs to the class of organic compounds known as long-chain 3-oxoacyl coas. (hmdb.ca)
  • Catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. (abcam.com)
  • Long-chain fatty acyl-CoA ligase is critical enzyme processing long-chain fatty acid acylation which is essential for lysophosphatidylinositol (lyso-PI) incorporation into glycosyl phosphatidylinositols (GPIs) [ 16 , 17 ]. (hindawi.com)
  • De novo sphingolipid biosynthesis starts with the condensation of serine and the product of long-chain fatty acyl-CoA ligase. (hindawi.com)
  • L. major preferentially incorporates myristoyl-CoA (C14) over palmitoyl-CoA (C16) into their long-chain base [ 19 , 20 ]. (hindawi.com)
  • This selection of specific long-chain fatty acyl-CoA reflects the presence of myristoyl-specific long-chain fatty acyl-CoA ligase in Leishmania [ 21 ]. (hindawi.com)
  • Through early identification, newborn screening provides an opportunity for treatment and significant reductions in morbidity and mortality ( 2 , 3 ). (cdc.gov)
  • A-B. Densitometry of MTT reduction in EV and HSD10 ov cells treated with A) 0, 0.1, 0.25, 0.5, 0.75, and 1 mM H2O2 (n = 3 for all groups), and B) 0, 0.1, and 0.25 mM TBH for 24 hours (n = 3 for all groups). (nih.gov)