Alpha-1,4-glucosidaseAlkalineLipaseGlucose-6-phosphataMitochondrialLipidAutosomal recessiveHepaticCytosolACP2Amino acidsGeneMutationsMembraneBiochemicalDeficiencyIntracellularProteinCathepsinMarker enzymesMicrosomalMembranesSerumFractionRatsMaltaseLysosomesFamilialAutophagyMacrophagesOxidaseActivityAbnormalProteinsPompeMyocardialSynthesisIncreasesNomenclatureGlycogen storesMetabolic disordersCarbohydratePathwaySuccinateSubunitTissueActivitiesDiseasesLevelsHepatocytesDistinctReactionsUltimately
Alpha-1,4-glucosidase1
- Its clinical presentation clearly differs from other forms of GSD, because it is caused by the deficiency of the lysosomal enzyme, alpha-1,4-glucosidase, leading to the pathologic accumulation of normally structured glycogen within the lysosomes of most tissues, differs Three forms of the disease exist: infantile-onset, late-onset juvenile and adult onset. (medscape.com)
Alkaline6
- In the present study, glucose, acid and alkaline phosphatases (ACP and ALP), α-amylase and lipase were measured for the first time in tissue homogenates of Biomphalaria arabica snails, molluscan intermediate host for Schistosoma mansoni in Saudi Arabia. (scialert.net)
- 2005). Carbohydrate metabolizing enzymes (e.g., α-amylase), lipid degrading enzymes (e.g., lipase) and phosphatases (acid and alkaline phosphatases) were recorded among target enzymes which should be disturbed and may provide more accurate information on the molluscicide-induced stress on molluscs. (scialert.net)
- 3. The plasma membrane enzyme, alkaline phosphatase, increased in activity after bile-duct ligation. (portlandpress.com)
- The purpose of this study was to investigate the activities of the total acid phosphatase (TAP), tartrate-resistant acid phosphatase (TRAP), low molecular weight protein tyrosine phosphatase (LMW-PTP) and alkaline phosphatase (ALP) enzymes, as well as the possible correlation in the serum and in unstimulated whole saliva of children. (bvsalud.org)
- Alkaline phosphatase. (bvsalud.org)
- Congenital hypophosphatasia is absence or low levels of serum alkaline phosphatase due to mutations in the gene encoding tissue-nonspecific alkaline phosphatase (TNSALP). (msdmanuals.com)
Lipase1
- In the blood serum the activity of cathepsins D and L, alanine aminopeptidase, acid phosphatase, lysosomal lipase and lysosomal esterase was determined. (nel.edu)
Glucose-6-phosphata6
- Glucose 6-phosphatase activity and ribonucleic acid, which are localized predominantly in the rough endoplasmic reticulum, were increased. (portlandpress.com)
- One form, Von Gierke disease ( GSD type Ia , glucose-6-phosphatase deficiency ), causes clinically significant end-organ disease with significant morbidity. (medscape.com)
- Glucose-6-phosphatase (G-6-P) is the specific enzyme deficiency in Von Gierke disease. (medscape.com)
- Glycogen storage disease (GSD) type I, also known as von Gierke disease, is a group of inherited autosomal recessive metabolic disorders of the glucose-6- phosphatase system which helps maintain glucose homeostasis. (medscape.com)
- [ 1 ] In 1952, Cori and Cori demonstrated that glucose-6-phosphatase (G6Pase) deficiency was a cause of GSD type I. (medscape.com)
- These autosomal recessive diseases are caused, respectively, by defects in the enzymes glucose-6-phosphatase and glucose-6-phosphatase translocase. (pediagenosis.com)
Mitochondrial5
- The activity of hepatic drug-metabolizing enzymes in post-mitochondrial fraction and the cytochrome P 450 contents in microsomal fraction decreased significantly, while lysosomal enzymes such as acid phosphatase, acid ribonuclease and cathepsin B showed an increase in the liver homogenates of infected animals. (ias.ac.in)
- 4. No changes in mitochondrial enzyme activities were noted after 1 day but there was a 50% reduction 8 days after ligation. (portlandpress.com)
- 7. Serum activities of mitochondrial, plasma membrane, microsomal, lysosomal and cytosol marker enzymes tended to increase post-ligation, particularly at 8 days. (portlandpress.com)
- 8. Monoamine oxidase, a predominantly mitochondrial enzyme, was greatly elevated in the serum after 1 day but had returned to normal activities by 8 days. (portlandpress.com)
- The AMPK consists of a catalytic α-subunit and the regulatory β- and γ-subunits that form a functionally active αβγ-heterotrimeric complex, and is widely distributed in all subcellular compartments (cytoplasmic, lysosomal, mitochondrial, and nuclear). (encyclopedia.pub)
Lipid3
- Lipid peroxidation damages primary and secondary lysosomal membranes, causing the release of acid phosphatases contained in lysosomes as hydrolytic enzymes and causing damage to vital organelles such as cellular and superficial granule membranes, and methionine fights these damages through multiple pathways. (creative-proteomics.com)
- Utsab Subedi presented "Regulation of lysolipid Signaling by lipid phosphate Phosphatase in vascular injury' moderated poster at industry day held at LSU Health Shreveport on October 11, 2019. (lsuhs.edu)
- Niemann-Pick disease is caused by an abnormal lysosomal lipid enzyme degradation system, and it is therefore considered to be a lysosomal storage disease. (pediagenosis.com)
Autosomal recessive2
- It is an autosomal recessive disorder in which there is an AGL gene mutations which causes deficiency in glycogen debranchinging enzyme and limited storage of dextrin. (medscape.com)
- Galactosemia is a rare autosomal recessive disorder that results from a defect in the enzyme galactose-1- phosphate uridyltransferase. (pediagenosis.com)
Hepatic2
- The activities of hepatic lysosomal enzymes were determined in rats after the acute administration of ethanol and its feeding for periods of 1 and 6 months. (johnshopkins.edu)
- In addition, hepatic enzyme levels were also determined. (biomedcentral.com)
Cytosol2
- 6. Lactate dehydrogenase, a cytosol enzyme, showed a decrease in activity after 1 day but an increase in tissue activities 8 days after ligation. (portlandpress.com)
- Immunohistochemical staining of cathepsin D in ischemic tissue sections revealed apparent diffusion of the enzyme from discrete organelles into the surrounding cytosol by 30 minutes after occlusion, but not at 15 minutes. (elsevierpure.com)
ACP22
- Lysosomal acid phosphatase is an enzyme that in humans is encoded by the ACP2 gene. (wikipedia.org)
- Acid phosphatase deficiency is caused by mutations in the ACP2 (beta subunit) and ACP3 (alpha subunit) genes. (wikipedia.org)
Amino acids3
- After the degradation of damaged proteins and lipids, amino acids and fatty acids are released into the cytoplasm and recycled for new biosynthesis of cellular components or energy production [ 4 ]. (hindawi.com)
- Methionine, one of the 20 amino acids that make up proteins, is an essential and ketogenic amino acid for mammals and plays a critical role in many cellular processes, including protein synthesis, DNA methylation and cellular redox homeostasis. (creative-proteomics.com)
- [5] The mechanism by which the glucosidase cleaves the α -1,6-linkage is not fully known because the amino acids in the active site have not yet been identified. (wikidoc.org)
Gene3
- Therapies may include a restrictive diet, dietary supplements, and toxin-removal procedures, as well as enzyme replacement, gene transfer, or organ transplantation. (newworldencyclopedia.org)
- Type A and type B disease are similar in that the ASM gene, which encodes the acid sphingomyelinase enzyme, is mutated. (pediagenosis.com)
- This enzyme has a molecular mass of 73.6 kDa, and is coded for by the gene glgX. (wikidoc.org)
Mutations2
- For practical purposes, depending on the enzyme activity and the presence of mutations in the G6Pase and T genes, respectively, GSD type I may be subdivided into 2 major forms. (medscape.com)
- [1] When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen storage disease type III can result. (wikidoc.org)
Membrane2
- As ascertained by plasma membrane marker enzyme activity, the main contaminant could be the plasma membrane components. (rupress.org)
- However, cytochemical tests for 5'AMPase and for acid phosphatase suggest that a large part of the plasma membrane marker enzyme activity present in the purified lysosome preparation could be associated with the lysosomal membrane. (rupress.org)
Biochemical6
- Impairment of these enzymes could be effective in affecting the feeding and reproductive competence at the mollusc population level (fertility, fecundity and reproductive rate).The effect of sublethal concentrations of different synthetic and natural molluscicide agents on reproduction and biochemical aspects of schistosome snail hosts is recently encouraged (Abdel Kader et al . (scialert.net)
- Biochemical assay for enzyme activity is the method of definitive diagnosis. (medscape.com)
- and (3) biochemical evidence of increased lysosomal fragility precedes anatomical evidence of enzyme translocation in the tissue. (elsevierpure.com)
- Cellular metabolism consists of numerous interconnected pathways that are catalyzed by enzymes in a series of stepwise biochemical reactions. (newworldencyclopedia.org)
- Cellular metabolism, involving the synthesis and breakdown of complex organic molecules, requires harmonious coordination of the various enzymes, biochemical reactions, and so forth. (newworldencyclopedia.org)
- In 1908, physician Sir Archibald Garrod coined the term "inborn errors of metabolism" to suggest that defects in specific biochemical pathways were due to an inadequate supply or a lack of a given enzyme. (newworldencyclopedia.org)
Deficiency6
- Deficiency of lysosomal acid phosphatase. (wikipedia.org)
- Enzyme deficiency results in glycogen accumulation in tissues. (medscape.com)
- Although at least 14 unique GSDs are discussed in the literature, the 4 that cause clinically significant muscle weakness are Pompe disease ( GSD type II , acid maltase deficiency), Cori disease ( GSD type III , debranching enzyme deficiency), McArdle disease ( GSD type V , myophosphorylase deficiency), and Tarui disease ( GSD type VII , phosphofructokinase deficiency). (medscape.com)
- Acid maltase deficiency is a unique glycogenosis since glycogen accumulation is lysosomal rather than in the cytoplasm. (medscape.com)
- GSD type II, also known as alpha glucosidase deficiency (GAA, acid maltase deficiency) or Pompe disease, is a prototypic lysosomal disease. (medscape.com)
- GSD type IV, also known as amylopectinosis, Glycogen Branching enzyme deficiency (GBE) or Andersen disease, is a rare disease that leads to early death. (medscape.com)
Intracellular3
- Autophagy is a cellular catabolic process that eliminates damaged cell organelles, unfolded proteins, and various intracellular pathogens through lysosomal degradation. (hindawi.com)
- Autophagy is an evolutionarily conserved cellular degradation pathway involving the digestion of intracellular components via the lysosomal pathway. (mdpi.com)
- Intracellular lectins and glycan-modifying enzymes mediate autophagy and control host immunity and inflammation. (nature.com)
Protein5
- Protein phosphatases reverse the covalent modifications of numerous cellular proteins imposed by the activation of protein kinases and, therefore, play key role in cell signaling metabolism, growth and differentiation. (bvsalud.org)
- At the lysosomal surface, free aldolase promotes the formation of a multiprotein complex, including the vacuolar H + -ATPase and the scaffold protein AXIN, and this complex ensures the effective binding between AMPK and LKB1, thereby activating AMPK [ 25 ] [ 26 ] . (encyclopedia.pub)
- A negative regulator of AMPK is the protein phosphatase 2C (PP2C), which dephosphorylates and inactivates the α-subunit of AMPK, causing the dissociation of the αβγ-heterotrimeric complex. (encyclopedia.pub)
- [9] A second protein, referred to as debranching enzyme, performs α-1,6-glucose cleavage. (wikidoc.org)
- Nasu, S., Wicks, F.D. and Gholson, R.K. L -Aspartate oxidase, a newly discovered enzyme of Escherichia coli , is the B protein of quinolinate synthetase. (enzyme-database.org)
Cathepsin4
- Lysosomal enzymes acid phosphatase, acid DNAase, acid RNAase cathepsin D,beta-galactosidase beta-glucuronidase were studied in mice and rat liver tissue within 3 days beginning from 4 o'clock a.m. at 4 hrs intervals. (msk.ru)
- Activities of acid phosphatase, acid RNA ase in mice liver tissue and of cathepsin D in rat and mice liver tissues was higher at morning and day time than at evening and night. (msk.ru)
- Total activities of cathepsin D, N-acetyl-β,D-glucosaminidase, and acid phoshphatase in ischemic tissue were not altered over the ensuing 2-hour period, but significant changes developed in the distribution of the enzymes between sedimentable (particle-bound) and nonsedimentable (soluble) fractions of the tissue homogenate. (elsevierpure.com)
- Unlike cathepsin D and glucosaminidase, changes in acid phosphatase were maximal by 45 minutes, after which further increases in nonsedimentable activity were not apparent. (elsevierpure.com)
Marker enzymes3
- 1. Marker enzymes for the principal subcellular organelles of rat liver were asayed in the liver of rats 1 day and 8 days after bile-duct ligation or after laparotomy as a control procedure. (portlandpress.com)
- J. 60: 604-617), was subfractionated by isopycnic centrifugation in a metrizamide gradient and the distribution of several marker enzymes was established. (rupress.org)
- As inferred from the relative specific activity of marker enzymes, it can be estimated that lysosomes are purified between 66 and 80 times in this fraction. (rupress.org)
Microsomal1
- 2. The microsomal enzymes in liver tissue showed complex changes. (portlandpress.com)
Membranes1
- Within the second half of a day the enzymatic activity redistributed in the cells with an increase of the activity in non-sedimenting fraction, caused by labilization of lysosomal membranes. (msk.ru)
Serum4
- Observing the changes of activity of some lysosomal enzymes in blood serum of female rabbits subjected to injection of 10 microg of ghrelin/kg of body weight. (nel.edu)
- As a result of ghrelin injection the activity of all the enzymes examined in blood serum increased markedly. (nel.edu)
- Changes of lysosomal enzymes activities in the blood serum caused by the effects of ghrelin should be regarded as the response of the lysosomal system. (nel.edu)
- The enzymatic activities of all the studied enzymes were higher in serum than in saliva. (bvsalud.org)
Fraction1
- By contrast the feeding of ethanol for a period of 6 months resulted in increases in the total activities of β-glucuronidase, β-N-acetylglucosaminidase and arylsulfatase A measured in the homogenates, and in the specific activities of these enzymes plus acid phosphatase measured in the lysosomal fraction. (johnshopkins.edu)
Rats1
Maltase1
- Acid maltase catalyzes the hydrogenation reaction of maltose to glucose. (medscape.com)
Lysosomes1
- In both cases, as ascertained by the enzyme distributions, the lysosomes are well separated from the peroxisomes. (rupress.org)
Familial1
- For example, in familial hypercholesterolemia, enzymes do not receive the signals that typically inhibit cholesterol synthesis, so that excessive production of cholesterol occurs, leading to early coronary vascular disease and strokes in patients. (newworldencyclopedia.org)
Autophagy1
- Autophagy is a conserved catabolic process that eliminates dysfunctional cytosolic biomolecules through vacuole-mediated sequestration and lysosomal degradation. (hindawi.com)
Macrophages2
- This analysis reveals that the RN is composed largely of strongly HLA-DR+, ATP- macrophages which contain lysosomal enzymes (ACP) in large amounts. (nih.gov)
- by contrast, interleukin-4 (IL-4)-activated macrophages mainly use oxidative phosphorylation and fatty acid oxidation to generate energy. (nature.com)
Oxidase1
- 5. Liver catalase and d-amino acid oxidase activities showed a slight increase at 1 day post-ligation but a significant fall by 8 days. (portlandpress.com)
Activity5
- Low-intensity physical activity is shown not to influence the activity of hepatocytes' enzymes and their proliferation. (peertechzpublications.com)
- The purpose of this work was to study the influence of physical work on the activity of hepatocytes' enzymes and their proliferation. (peertechzpublications.com)
- A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen , which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. (wikidoc.org)
- [10] Activity of the two enzymes is not always necessarily coupled. (wikidoc.org)
- Interaction with C4 dicarboxylic acids and identification of a novel L -aspartate: fumarate oxidoreductase activity. (enzyme-database.org)
Abnormal2
- in the absence of a particular enzyme, the abnormal or unregulated synthesis of complex molecules might result. (newworldencyclopedia.org)
- inborn errors of metabolism are caused by mutant genes that produce abnormal enzymes whose function is altered. (newworldencyclopedia.org)
Proteins1
- Proteins that catalyze both functions are referred to as glycogen debranching enzymes (GDEs). (wikidoc.org)
Pompe1
- These inherited enzyme defects usually present in childhood, although some, such as McArdle disease and Pompe disease, have separate adult-onset forms. (medscape.com)
Myocardial1
- Dr. Manikandan Panchatcharam (PI) and his co-investigators, Drs. Sumitra Miriyala and Hong Sun, were recently awarded an R21 for the NIH grant entitled ' Alcohol mediated myocardial lysophosphatidic acid signaling' from the National Institute of Alcohol Abuse and Alcoholism. (lsuhs.edu)
Synthesis3
- Six major pathways have been studied in immune cells in detail: glycolysis, the tricarboxylic acid (TCA) cycle, the pentose phosphate pathway, fatty acid oxidation, fatty acid synthesis and amino acid metabolism. (nature.com)
- Here, we provide a brief refresher course on six of the major metabolic pathways involved (specifically, glycolysis, the tricarboxylic acid (TCA) cycle, the pentose phosphate pathway, fatty acid oxidation, fatty acid synthesis and amino acid metabolism), giving specific examples of how precise changes in the metabolites of these pathways shape the immune cell response. (nature.com)
- Figure 5: Fatty acid synthesis and oxidation in immunity. (nature.com)
Increases3
- In addition, there were increases in the free activities of β-glucuronidase and acid phosphatase measured in the homogenates. (johnshopkins.edu)
- The increases in lysosomal enzymes were accompanied by histologic changes consisting only of mild fatty infiltration. (johnshopkins.edu)
- The most favorable is moderate load, which leads to the activation of oxidative-reducing enzymes and increases the number of binucleated hepatocytes, which persist for a long time after the end of the experiment. (peertechzpublications.com)
Nomenclature2
- Glucosyltransferase and glucosidase are performed by a single enzyme in mammals, yeast, and some bacteria, but by two distinct enzymes in E. coli and other bacteria, complicating nomenclature. (wikidoc.org)
- The entries below are proposed additions and amendments to the Enzyme Nomenclature list . (enzyme-database.org)
Glycogen stores1
- Glycogen debranching enzymes assist phosphorylase, the primary enzyme involved in glycogen breakdown , mobilize glycogen stores. (wikidoc.org)
Metabolic disorders1
- Metabolic disorders typically result when an enzyme necessary for some step in a metabolic process is missing or improperly constructed due to a genetic defect. (newworldencyclopedia.org)
Carbohydrate1
- With an enzyme defect, carbohydrate metabolic pathways are blocked, and excess glycogen accumulates in affected tissues. (medscape.com)
Pathway1
- The enzyme participates in the menthol-biosynthesis pathway of Mentha plants. (enzyme-database.org)
Succinate2
- Metabolites, such as succinate and citrate, and enzymes, such as pyruvate kinase isoenzyme M2 (PKM2), glyceraldehyde 3-phosphate dehydrogenase (GAPDH) and enolase, have roles outside of metabolism that promote specific events during immune cell activation. (nature.com)
- The enzyme is a member of the succinate dehydrogenase/fumarate-reductase family of enzymes [5]. (enzyme-database.org)
Subunit1
- cooling work PI3K and changes car, the HSV-1 activator serves needed by the MRN blood( MRE11A: RAD50: NBN) and RBBP8( CtIP) considered with BRCA1: BARD1, and coupled by EXO1 or DNA2 in subunit with DNA enzymes BLM, WRN and BRIP1( BACH1)( Sartori et al. (familie-vos.de)
Tissue2
- Lysosomal enzyme activities did not change in the liver tissue. (portlandpress.com)
- an enzyme defective within a particular organ or tissue, such as the liver , muscle , or brain , might lead to low energy production or prevent transport to the part of the body requiring energy. (newworldencyclopedia.org)
Activities2
- Effect of ghrelin on activities of some lysosomal hydrolases in rabbits. (nel.edu)
- most enzyme activities of the calf small intestine. (123dok.net)
Diseases1
- Moderate overexpression of PSAP is also characteristic of diseases of the bone (such as Paget's disease or hyperparathyroidism), diseases of blood cells (such as sickle-cell disease), multiple myeloma, or lysosomal storage diseases (such as Gaucher's disease). (attogene.com)
Levels1
- the substrate typically catalyzed by the enzyme might accumulate to toxic levels. (newworldencyclopedia.org)
Hepatocytes1
- Note the intensively stained vacuoles in the hepatocytes (periodic acid-Schiff, original magnification X 27). (medscape.com)
Distinct4
- Lysosomal acid phosphatase is composed of two subunits, alpha and beta, and is chemically and genetically distinct from red cell acid phosphatase. (wikipedia.org)
- Lysosomal acid phosphatase 2 is a member of a family of distinct isoenzymes which hydrolyze orthophosphoric monoesters to alcohol and phosphate. (wikipedia.org)
- When glucosyltransferase and glucosidase are catalyzed by distinct enzymes, "glycogen debranching enzyme" usually refers to the glucosidase enzyme . (wikidoc.org)
- In E. coli and other bacteria, glucosyltransferase and glucosidase functions are performed by two distinct enzymes. (wikidoc.org)
Reactions1
- These enzymes normally catalyze reactions that ultimately convert glycogen compounds to glucose. (medscape.com)
Ultimately1
- Lysosomal acid phosphatase increased during early metamorphosis, and ultimately the bulk of the cytoplasm was destroyed in autophagic vacuoles. (montclair.edu)