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Encyclopedia results for Pyruvate kinase deficiency

Pyruvate kinase deficiency





Encyclopedia results for Pyruvate kinase deficiency

  1. Pyruvate kinase deficiency

    Refimprove date August 2008 Infobox Disease Name Pyruvate kinase deficiency Image Phosphoenolpyruvic acid.svg Caption Phosphoenolpyruvate DiseasesDB 11090 ICD10 ICD10 D 55 2 d 55 ICD9 ICD9 282.3 ICDO OMIM 266200 MedlinePlus 001197 eMedicineSubj med eMedicineTopic 1980 MeshID Pyruvate kinase deficiency , also called erythrocyte pyruvate kinase deficiency , ref OMIM 266200 ref is an heredity inherited metabolic disorder of the enzyme pyruvate kinase which affects the survival of red blood cell s and causes them to deform into echinocytes on peripheral blood smears. Both autosome autosomal Dominance genetics dominant ref cite journal pmid 6475936 year 1984 month October author Etiemble, J Picat, C Dhermy, D Buc, Ha Morin, M Boivin, P title Erythrocytic pyruvate kinase deficiency and hemolytic anemia inherited as a dominant trait volume 17 issue 3 pages 251 60 issn 0361 8609 journal American journal of hematology doi 10.1002 ajh.2830170305 ref and recessive ref cite journal pmid 11186276 date 1 December 2000 author Carey, Pj Chandler, J Hendrick, A Reid, Mm Saunders, Pw Tinegate, H Taylor, Pr West, N title Prevalence of pyruvate kinase deficiency in northern European population in the north of England. Northern Region Haematologists Group volume 96 issue 12 pages 4005 6 issn 0006 4971 journal Blood url http www.bloodjournal.org cgi pmidlookup?view long&pmid 11186276 format Free full ... is autosomal recessive. Pyruvate kinase deficiency is the second most common cause of enzyme deficient hemolytic anemia , following G6PD deficiency . Causes A variety of mutations can lead to lowered production, activity, or stability of pyruvate kinase, an enzyme essential to glycolysis . A total ... triphosphate ATP through glycolysis . A deficiency in pyruvate kinase, the enzyme that potentiates the last step of glycolysis phosphoenolpyruvate converted to pyruvate , results in red blood cells ... Inborn errors of carbohydrate metabolism DEFAULTSORT Pyruvate Kinase Deficiency Category Inborn errors ...   more details



  1. Pyruvate kinase

    through metabolic pathways. Deficiency Genetic defects of this enzyme cause the disease known as pyruvate kinase deficiency . In this condition, a lack of pyruvate kinase slows down the process of glycolysis ... cycle is not available. One example is red blood cells , which in a state of pyruvate kinase deficiency rapidly become deficient in ATP and can undergo hemolysis . Therefore, pyruvate kinase deficiency can cause hemolytic anemia . Role in gluconeogenesis Pyruvate kinase also serves as a regulatory ...Image pkb.jpg thumb right 250px Typical Pyruvate Kinase Structure, small X ray Crystallography Derived small Pyruvate kinase List of EC numbers EC 2 EC 2.7.1 Phosphotransferases with an Alcohol Group as Acceptor ... of pyruvate and one molecule of adenosine triphosphate ATP . Reaction The reaction with pyruvate kinase Image Pyruvate kinase.png left thumb This process also requires a magnesium ion. The enzyme is a transferase ... dehydrogenase to reduce pyruvate to lactate. In humans, there are two pyruvate kinase isozymes ..., and are, in general, irreversible under wild type conditions . Pyruvate kinase activity is regulated ... pyruvate kinase is also regulated indirectly by epinephrine and glucagon , through protein kinase A . This protein kinase phosphorylates liver pyruvate kinase to deactivate it. Muscle pyruvate kinase ... phosphatase I, leading to dephosphorylation and activation of pyruvate kinase. These controls prevent pyruvate kinase from being active at the same time as the enzymes that catalyze the reverse ... and other substrates. When pyruvate kinase is inhibited by phosphorylation which occurs in the fasting ... will have both Pyruvate kinase and PPDK. ref citation doi 10.1128 EC.00258 06 pmc 1694820 year ... External links MeshName Pyruvate kinase glycolysis Kinases Glycolysis enzymes Category EC 2.7.1 de Pyruvatkinase es Piruvato quinasa fr Pyruvate kinase it Piruvato chinasi nl Pyruvaatkinase pl ... pathway, which produces pyruvate molecules, the final product of aerobic glycolysis. However ...   more details



  1. Pyruvate carboxylase deficiency

    Unreferenced date December 2007 Infobox Disease Name Pyruvate carboxylase deficiency Image Oxaloacetic acid.png Caption Oxaloacetate is the product of pyruvate carboxylase DiseasesDB 7378 ICD10 ICD10 E 74 4 e 70 ICD9 ICD9 271.8 ICDO OMIM 266150 MedlinePlus eMedicineSubj med eMedicineTopic 1979 eMedicine mult eMedicine2 ped 1967 MeshID D015324 Pyruvate carboxylase deficiency is an inherited disorder that causes lactic acid and other potentially toxic compounds to accumulate in the blood . High levels .... Pyruvate carboxylase deficiency is a rare condition, with an estimated incidence of 1 in 250,000 births ... at least three types of pyruvate carboxylase deficiency, which are distinguished by the severity ... or periods without food. Children with pyruvate carboxylase deficiency type A typically survive only into early childhood. Type B Pyruvate carboxylase deficiency type B has life threatening signs ... thumb right Pyruvate carboxylase deficiency has an Recessive gene autosomal recessive pattern of inheritance. Mutations in the PC gene cause pyruvate carboxylase deficiency. The PC gene provides instructions for making an enzyme called pyruvate carboxylase . This enzyme is active in mitochondria ... to the neurologic features of pyruvate carboxylase deficiency. This condition is inherited in an autosomal ... bookshelf br.fcgi?book gene&part pdc GeneReview NCBI NIH UW entry on Pyruvate Carboxylase Deficiency See also NLM pyruvatecarboxylasedeficiency Mitochondrial diseases Carbohydrate metabolic pathology DEFAULTSORT Pyruvate Carboxylase Deficiency Category Autosomal recessive disorders .... Pyruvate carboxylase also plays a role in the formation of the protective sheath that surrounds certain ... gene reduce the amount of pyruvate carboxylase in cells or disrupt the enzyme s activity. The missing ... the body s ability to make energy in mitochondria. Additionally, a loss of pyruvate carboxylase allows .... Researchers suggest that the loss of pyruvate carboxylase function in the nervous system, particularly ...   more details



  1. Pyruvate dehydrogenase deficiency

    Infobox Disease Name Pyruvate dehydrogenase deficiency Image Caption DiseasesDB 30060 ICD10 ICD10 E 74 4 e 70 ICD9 ICD9 271.8 ICDO OMIM 312170 MedlinePlus eMedicineSubj ped eMedicineTopic 1969 MeshID D015325 Pyruvate Dehydrogenase Deficiency PDH is a human genetic disease. Inheritance and pathophysiology It most commonly follows an X linked inheritance pattern, but is approximately equally prevalent in both males and females. This is because heterozygote females commonly manifest severe symptoms, an unusual situation in X linked conditions. Due to the genes involved being essential to life, some of the genetic causes are extremely rare, in the order of 1 in millions. It affects a gene which codes for a critical enzyme complex, the Pyruvate dehydrogenase complex PDC which links the metabolic pathways of glycolysis and the citric acid cycle by transforming pyruvate into Acetyl CoA The pyruvate dehydrogenase complex facilitates oxidative decarboxylation , the chemical reaction between glycolysis and the citric acid cycle . Deficiencies of the regulatory complex pyruvate dehydrogenase phosphatase have also been investigated. ref name pmid16574315 cite journal author Maj MC, Cameron JM, Robinson BH title Pyruvate dehydrogenase phosphatase deficiency orphan disease or an under diagnosed condition? journal Mol. Cell. Endocrinol. volume 249 issue 1 2 pages 1 9 year 2006 month April pmid 16574315 doi 10.1016 j.mce.2006.02.003 url http linkinghub.elsevier.com retrieve pii S0303 7207 06 00051 7 ref Presentation PDH causes Lactic acidosis large amounts of lactic acid in the blood but with a normal pyruvate lactic acid lactate ratio. Symptoms are varied, and include developmental defects especially of the brain and nervous system , muscular spasticity and early death. Genetics PDH deficiency is most commonly linked to the alpha unit of Pyruvate dehydrogenase E1 , which is X linked ... PW title Therapeutic potential of dichloroacetate for pyruvate dehydrogenase complex deficiency ...   more details



  1. (pyruvate kinase)-phosphatase

    enzyme Name pyruvate kinase phosphatase EC number 3.1.3.49 CAS number 79986 25 7 IUBMB EC number 3 1 3 49 GO code 0050408 image width caption In enzymology , a pyruvate kinase phosphatase EC number 3.1.3.49 is an enzyme that catalysis catalyzes the chemical reaction pyruvate kinase phosphate H sub 2 sub O math rightleftharpoons math pyruvate kinase phosphate Thus, the two substrate biochemistry substrates of this enzyme are pyruvate kinase phosphate and water H sub 2 sub O , whereas its two product chemistry products are pyruvate kinase and phosphate . This enzyme belongs to the family of hydrolase s, specifically those acting on phosphoric ester monoester bonds. The systematic name of this enzyme class is ATP pyruvate 2 O phosphotransferase phosphate phosphohydrolase . This enzyme is also called pyruvate kinase phosphatase . References reflist 1 cite journal author Jett MF, Hue L, Hers HG date 1981 title Pyruvate kinase phosphatase journal FEBS. Lett. volume 132 pages 183&ndash 6 pmid 6271587 doi 10.1016 0014 5793 81 81156 8 issue 2 DEFAULTSORT Pyruvate Kinase Phosphatase Category EC 3.1.3 Category Enzymes of unknown structure hydrolase stub ...   more details



  1. Pyruvate dehydrogenase kinase

    Pyruvate dehydrogenase kinase also pyruvate dehydrogenase complex kinase , PDC kinase , or PDK EC number 2.7.11.2 is a kinase enzyme which acts to inactivate the enzyme pyruvate dehydrogenase by phosphorylate phosphorylating it using Adenosine triphosphate ATP . PDK thus participates in the regulation of the pyruvate dehydrogenase complex of which pyruvate dehydrogenase is the first component. Both PDK and the pyruvate dehydrogenase complex are located in the mitochondrial matrix of eukaryote s. The complex acts to convert pyruvate a product of glycolysis in the cytosol to acetyl coA , which is then oxidized in the mitochondria to produce energy, in the citric acid cycle . By downregulating the activity of this complex, PDK will decrease the oxidation of pyruvate in mitochondria and increase the conversion of pyruvate to lactic acid lactate in the cytosol. The opposite action of PDK, namely the dephosphorylation and activation of pyruvate dehydrogenase, is catalyzed by a phosphoprotein phosphatase called pyruvate dehydrogenase phosphatase . Pyruvate dehydrogenase kinase should not be confused with Phosphoinositide dependent kinase 1 , which is also sometimes known as PDK1 . Genes PDK has four isozymes PDK1 PDK2 PDK3 PDK4 Regulation Pyruvate dehydrogenase kinase is stimulated by Adenosine triphosphate ATP , NADH and acetyl CoA . It is inhibited by Adenosine diphosphate ADP , NAD , CoA SH and pyruvate . Pyruvate dehydrogenase kinase is also inhibited by the drug dichloroacetate which has been tried as a treatment of certain metabolic diseases and is under investigation as a treatment of cancer. External links MeshName pyruvate dehydrogenase kinase EC number 2.7.11.2 Serine threonine specific protein kinases Citric acid cycle enzymes Category EC 2.7.11 Category Citric acid cycle Category Glycolysis enzyme stub es Piruvato deshidrogenasa kinasa id Kinase piruvat dehidrogenase pt Piruvato desidrogenase quinase ...   more details



  1. Glycerol Kinase Deficiency

    Userspace draft source ArticleWizard date March 2011 Causes Glycerol Kinase Deficiency GKD has two main causes associated with it. The first cause is isolated enzyme deficiency. The enzyme glycerol kinase ... glycerol kinase deficiency in the mouse leads to growth retardation, altered fat metabolism, autonomous ... Kinase Deficiency Molecular Genetic Evidence for Deletions. American Journal of Human Genetics ... ref Glycerol Kinase Deficiency s effect on Glycolysis In order to understand how this condition affects ... Kinase Deficiency. When a human s body needs to use stored fat for energy it will release glycerol ... and can cause serious damage if left untreated. Symptoms Glycerol Kinase Deficiency causes the condition ..., and Bwee Tien Poll The. Glycerol Kinase Deficiency Residual Activity Explained by Reduced Transcription ... Kinase Deficiency. Small Molecule Pathway Database. 6 Mar. 2010. Web. 24 Mar. 2011. http pathman.smpdb.ca ... Wishart, David. Glycerol Kinase Deficiency. Small Molecule Pathway Database. 6 Mar. 2010. Web. 24 ... in Glycerol Kinase Deficiency. Biochemical Medicine 33.2 1985 189 99. Print. ref , learning ... common but does occur . Treatment Treatments for Glycerol Kinase Deficiency are targeted to treat ... with Glycerol Kinase Deficiency has varying degrees of symptoms and thereby requires different medicines ... GKD and it can be either symptomatic or asymptomatic. ref Lola, Rahib, et al. Glycerol kinase deficiency alters expression of genes involved in lipid metabolism, carbohydrate metabolism, and insulin .... In this deficiency the genotype is not associated with the phenotype. The presence of certain ... kinase gene present on the locus Xp21 of the X chromosome is either deleted or mutated. Females who ... taking in the sugar glucose and converting it into the energy rich molecule pyruvate . Inside the overall ... to be transformed into pyruvate. The glucose first gathers a phosphate group from an Adenosine ... glycerol kinase comes into the picture. The enzyme is used in the first step in turning ...   more details



  1. Mevalonate kinase deficiency

    Infobox Disease Name Mevalonate kinase deficiency Image Mevalonic.jpg Caption A patient with mevalonate kinase deficiency at the age of 21 months, displaying characteristic craniofacial features. DiseasesDB 29843 ICD10 ICD9 ICDO OMIM 251170 MedlinePlus eMedicineSubj eMedicineTopic MeshID D054078 Mevalonate kinase deficiency , also called mevalonic aciduria , ref name omim OMIM 251170 ref is an autosome autosomal Dominance genetics recessive metabolic disorder that disrupts the biosynthesis of cholesterol and isoprenoid s. ref name pmid8352861 cite journal author Mancini J, Philip N, Chabrol B, Divry P, Rolland MO, Pinsard N title Mevalonic aciduria in 3 siblings a new recognizable metabolic encephalopathy journal Pediatr. Neurol. volume 9 issue 3 pages 243 246 year 1993 pmid 8352861 doi 10.1016 0887 8994 93 90095 T month May Jun ref Diagnosis Mevalonate kinase deficiency causes an accumulation of mevalonic acid in the urine , resulting from insufficient activity of the enzyme mevalonate kinase ref name pmid17578678 cite journal author Bret n Mart nez JR, C novas Mart nez A, Casa a P rez S, Escrib Alepuz J, Gim nez V zquez F title Mevalonic aciduria report of two cases journal J. Inherit. Metab. Dis. volume 30 issue 5 pages 829 year 2007 month Oct pmid 17578678 doi 10.1007 s10545 007 0618 7 ref ATP mevalonate 5 phosphotransferase EC 2.7.1.36 . Image Mevalonate pathway.png 350px thumb center Mevalonate pathway The disorder was first described in 1985. ref name pmid4053401 cite journal author Berger R, Smit GP, Schierbeek H, Bijsterveld K, le Coultre R title Mevalonic aciduria an inborn error of cholesterol biosynthesis? journal Clin. Chim. Acta volume 152 issue 1 2 pages 219 ... error of metabolism , mevalonate kinase deficiency usually results in developmental delay , hypotonia ... to thrive and several other features. Image autorecessive.svg thumb right Mevalonate kinase deficiency ... bin Databases BIRX birx doc?phtomim 251170 Mevalonate kinase deficiency RareDiseases 3588 ...   more details



  1. Janus kinase 3 deficiency

    Janus kinase 3 deficiency or JAK3 deficiency is a defect in the body s cytokine receptors and their signaling. ref name Bolognia cite book author Rapini, Ronald P. Bolognia, Jean L. Jorizzo, Joseph L. title Dermatology 2 Volume Set publisher Mosby location St. Louis year 2007 pages 820 isbn 1 4160 2999 0 oclc doi accessdate 2009 12 17 ref JAK3 encodes Janus kinase 3, a tyrosine kinase that belongs to the Janus family. JAK3 functions in signal transduction and interacts with members of the STAT signal transduction and activators of transcription family. The cause of JAK3 deficiency. ref cite web url http www.ncbi.nlm.nih.gov sites entrez?Db gene&Cmd ShowDetailView&TermToSearch 3718 title JAK3 Janus kinase 3 Homo sapiens work NCBI publisher Department of Health & Human Services accessdate 17 December 2009 ref The deficiency causes the near absence of T lymphocytes and Natural killer cells and normal or elevated B lymphocytes due to an autosomal recessive variant of severe combined immunodeficiency SCID ref cite web url http bioinf.uta.fi xml idr ff FF9.xml title JAK3 deficiency work ImmunoDeficiency Resource IDR publisher IMT Bioinformatics accessdate 17 December 2009 ref See also List of cutaneous conditions References reflist Cutaneous condition stub Category Cytokine receptors Category Noninfectious immunodeficiency related cutaneous conditions ...   more details



  1. Dolichol kinase deficiency

    Infobox Disease Name PAGENAME Image Caption DiseasesDB ICD10 ICD9 ICDO OMIM 610768 MedlinePlus eMedicineSubj eMedicineTopic MeshID Dolichol kinase deficiency is a cutaneous condition caused by a mutation in the dolichol kinase gene. ref name Bolognia cite book author Rapini, Ronald P. Bolognia, Jean L. Jorizzo, Joseph L. title Dermatology 2 Volume Set publisher Mosby location St. Louis year 2007 pages isbn 1 4160 2999 0 oclc doi accessdate ref ref name pmid17273964 cite journal author Kranz C, Jungeblut C, Denecke J, et al. title A defect in dolichol phosphate biosynthesis causes a new inherited disorder with death in early infancy journal Am. J. Hum. Genet. volume 80 issue 3 pages 433 40 year 2007 month March pmid 17273964 pmc 1821118 doi 10.1086 512130 url http linkinghub.elsevier.com retrieve pii S0002 9297 07 60092 5 ref It is also known as Congenital disorder of glycosylation 1m. See also CEDNIK syndrome List of cutaneous conditions References reflist Dermatology stub Glycoproteinoses Category Genodermatoses ...   more details



  1. Il-1R?Associated Kinase 4 Deficiency

    orphan date August 2010 Members of interleukin 1 receptor Il 1R and the Toll like receptor superfamily share an intracytoplasmic Toll IL 1 receptor TLR domain, which mediates recruitment of the interleukin 1 interleukin 1 receptor associated kinase IRAK complex via TIR containing adapter molecules. ref Ku CL, Yang K, Bustamante J, et al. Inherited disorders of human Toll like receptor signaling immunological implications. Immunol Rev. 2005 203 10 20. ref Three unrelated, otherwise healthy children with recurrent pyogenic infections due to pneumococci and staphylococci had normal immunocompetence by standard immune studies. They had normal titers of antipneumococcal antibodies. However, their blood and fibroblast cells did not activate nuclear factor B and mitogen activated protein kinase and failed to induce downstream cytokines in response to any of the known ligands of TIR bearing receptors. Each was found to have an inherited deficiency of IRAK 4. Thus, the TIR IRAK signaling pathway appears to be crucial for protective immunity against specific bacteria but is redundant against most other microorganisms . ref Ku CL, Yang K, Bustamante J, et al. Inherited disorders of human Toll like receptor signaling immunological implications. Immunol Rev. 2005 203 10 20. ref References reflist Category Enzymes Category Genetic disorders with no OMIM genetics stub ...   more details



  1. File:Pyruvate kinase wpmp.png

    Move to Commons By Richard Wheeler Zephyris 2006. For the Wikipedia Wikiproject Metabolic Pathways metabolic pathways wikiproject. Licensing GFDL self with disclaimers migration relicense ...   more details



  1. Deficiency

    Wiktionary A deficiency is a lack of something. Example There is a deficiency of vitamin D in his diet In mathematics, a deficient number is a number n for which n     2 n . In medicine there are a variety of nutrient deficiencies Avitaminosis is a deficiency of vitamins. Boron deficiency medicine Chromium deficiency Iron deficiency medicine Iodine deficiency Magnesium deficiency medicine Micronutrient deficiency In construction , a deficiency is an item, or condition that is considered sub standard, or below minimum expectations, such as those mandated by either drawing s or specification s or the building code or the fire code, and or any combination of the foregoing. Deficiencies are routinely discussed and dealt with in construction site meeting s. In genetics, a genetic deletion is also called a Deficiency. In real estate law , a deficiency in the ability to pay off a debt is called a deficiency judgment or deficiency judgement. disambig ar de Mangel es Deficiencia nl Defici ntie no Mangel ...   more details



  1. Kinase

    pathway. Kinases are often named after their substrates. References Reflist 2 See also Protein kinase Cyclin dependent kinase Ca2 calmodulin dependent protein kinase Ca sup 2 sup calmodulin dependent protein kinase Signal transduction G protein coupled receptor Kinases Category EC 2.7.1 an Quinasa ca Quinasa cs Kin za de Kinase es Quinasa fa fr Kinase ko io Kinazo id Kinase it Chinasi he nl Kinase ja oc Quinasa pl Kinazy pt Cinase ru sr Kinaza fi Kinaasi sv Kinas uk vi Kinase zh ...   more details



  1. Pyruvate dehydrogenase

    Phosphorylation of E1 by pyruvate dehydrogenase kinase PDK inactivates E1 and subsequently the entire complex. This is reversed by pyruvate dehydrogenase phosphatase . Pyruvate dehydrogenase is stimulated ... , which are antigens recognized by anti mitochondrial antibodies . Pyruvate dehydrogenase PDH deficiency .... Pyruvate Dehydrogenase Complex Deficiency. EMedicine. 11 Dec. 2007. WebMD. 14 Dec. 2008 http ... Pyruvate dehydrogenase deficiency References reflist 2 External links MeshName Pyruvate Dehydrogenase ... width caption Crystallographic structure of pyruvate dehydrogenase PDH . PH is a six domain ... flop action of thiamin pyrophosphate dependent enzymes revealed by human pyruvate dehydrogenase journal ... doi 10.1002 jcc.20084 url ref Not to be confused with pyruvate decarboxylase , the enzyme which catalyses alcoholic fermentation . Pyruvate dehydrogenase E1 is the first component enzyme of pyruvate dehydrogenase complex PDC . The pyruvate dehydrogenase complex contributes to transforming pyruvate into acetyl CoA by a process called pyruvate decarboxylation . Acetyl CoA may then be used in the citric acid cycle to carry out cellular respiration , so pyruvate dehydrogenase contributes to linking ... adenine dinucleotide NADH . EC number 1.2.4.1 . Function Pyruvate dehydrogenase E1 performs the first two reactions within the pyruvate dehydrogenase complex PDC a decarboxylation of substrate 1 pyruvate ... px center alt Regulation of Pyruvate Dehydrogenase Mechanism File PyruvateDehydrgenaseMech1.gif Pyruvate ... the electrophilic ketone of pyruvate. The intermediate alkoxide then decarboxylation decarboxylates ... decarboxylates pyruvate, the acetyl portion becomes a hydroxyethyl derivative covalently attached to TPP ... revealed by human pyruvate dehydrogenase journal J. Biol. Chem. volume 278 issue 23 pages 21240 6 ... F, Guest JR, Furey W. title Structure of the pyruvate dehydrogenase multienzyme complex E1 component .... ref name pmid12651851 The active site for pyruvate dehydrogenase image created from PDB 1NI4 ...   more details



  1. Pyruvate carboxylase

    carboxylase deficiency deficiency of pyruvate carboxylase can cause lactic acidosis as a result ... of the enzyme deficiency, excess pyruvate is converted into lactate instead. As a key role of gluconeogenesis is in the maintenance of blood sugar , deficiency of pyruvate carboxylase can also ... NCBI NIH UW entry on Pyruvate Carboxylase Deficiency See also Pyruvate carboxylase deficiency ...FixBunching beg enzyme Name Pyruvate carboxylase EC number 6.4.1.1 CAS number 9014 19 1 IUBMB EC number 6 4 1 1 GO code 0004736 image Pyruvate Carboxylase fromPDB 2QF7.png width caption X ray crystallography Biological macromolecular crystallography Crystallographic structure of pyruvate carboxylase ..., mechanism and regulation of pyruvate carboxylase journal Biochem. J. volume 413 issue 3 pages 369 87 ... Pfam box Symbol PYR CT Name Pyruvate carboxyltransferase Pfam PF00682 InterPro IPR000891 PROSITE ... Pyruvate carboxylase image caption Symbol PC AltSymbols HGNCid 8636 Chromosome 11 Arm q Band 11 LocusSupplementaryData ... PDB FixBunching end Pyruvate carboxylase PC is an enzyme of the ligase class that catalyzes the depending on the species irreversible carboxylation of pyruvate to form oxaloacetate OAA . gallery ... pmid18613815 Pyruvate carboxylase was first discovered in 1959 by M. F. Utter and D. B. Keech. ref name pmid13840551 cite journal author Utter MF, Keech DB title Formation of oxaloacetate from pyruvate ... NG title A re examination of the electron microscopic appearance of pyruvate carboxylase from chicken ... of pyruvate carboxylase in mammalian tissues journal Cell. Mol. Life Sci. volume 63 issue 7 8 pages ... structure of chicken pyruvate carboxylase journal Biochem. Biophys. Res. Commun. volume 295 issue ... ME, Wallace JC title Identification of novel alternatively spliced pyruvate carboxylase mRNAs with divergent ... Biao J, Sueda S, Kondo H title Structure of the biotin carboxylase subunit of pyruvate carboxylase ... tetramer for S. aureus pyruvate carboxylase in complex with coenzyme A journal Structure volume ...   more details



  1. Pyruvate decarboxylation

    Pyruvate decarboxylation also known as the Naypyidaw Reaction , Swanson Conversion ref http www.rpi.edu dept bcbp molbiochem MBWeb mb1 part2 krebs.htm ref , link reaction or oxidative decarboxylation reaction is the Autocatalytic reaction Far from equilibrium far from equilibrium biochemical reaction that uses pyruvate to form acetyl CoA , releasing NADH , a reducing equivalent, and carbon dioxide via decarboxylation . It is known as the link reaction because it forms an important link between the metabolic pathway s of glycolysis and the citric acid cycle . This reaction is usually catalyzed by the pyruvate dehydrogenase complex as part of aerobic respiration . ref Alberts et al. Molecular Biology of the Cell. Garland Science, 2001. ISBN 0 8153 4072 9 ref In eukaryotes , pyruvate decarboxylation takes place exclusively inside the mitochondrial matrix in prokaryotes similar reactions take place in the cytoplasm and at the plasma membrane. ref Raven et al. Biology, 8th edition. McGraw Hill ... Reaction foward enzyme pyruvate dehydrogenase complex PDHC reverse enzyme substrate pyruvate Pyr product ... out in the mitochondria , unlike the reactions of glycolysis which are cytosol ic. The conversion of pyruvate to acetyl CoA by the pyruvate dehydrogenase complex is a key step in the liver in particular, as it removes any chance of conversion of pyruvate to glucose, or as a transamination substrate. It commits pyruvate to entering the citric acid cycle, where it is either used as a substrate for oxidative ... for fatty acid and isoprenoid biosynthesis. The oxidative decarboxylation of pyruvate in anaerobic ..., Juan C. Fontecilla Camps Crystal Structure of the Free Radical Intermediate of Pyruvate Ferredoxin ... of by the production of H sub 2 sub via hydrogenase. p To summarise p 1. Pyruvate is decarboxylated ... Cycle . References references External links http www.rahulgladwin.com blog 2007 01 notes on pyruvate metabolism.html Notes on Pyruvate decarboxylation http www.genome.jp kegg pathway.html KEGG ...   more details



  1. Pyruvate decarboxylase

    enzyme Name Pyruvate decarboxylase EC number 4.1.1.1 CAS number 9001 04 1 IUBMB EC number 4 1 1 1 GO code 0004737 image Pyruvate decarb 1.jpg width caption Reaction catalyzed by pyruvate decarboxylase br pyruvate thiamine pyrophasphate TPP hydroxyethyl TPP CO sub 2 sub Not to be confused with pyruvate dehydrogenase , the enzyme which catalyses the pyruvate decarboxylation link reaction . Pyruvate decarboxylase ... by fermentation. Pyruvate decarboxylase starts this process by converting pyruvate into acetaldehyde ... doi url accessdate ref Pyruvate decarboxylase depends on Cofactor biochemistry cofactors thiamine pyrophosphate TPP and magnesium. This enzyme should not be mistaken for the unrelated enzyme pyruvate dehydrogenase , an oxidoreductase EC number 1.2.4.1 , that catalyzes the oxidative decarboxylation of pyruvate to acetyl CoA . Yeast In yeast, pyruvate decarboxylase acts independently during wikt anaerobic .... Pyruvate decarboxylase creates the means of CO sub 2 sub elimination, which the cell dispels. The enzyme ... B, Jordan F title Catalytic centers in the thiamin diphosphate dependent enzyme pyruvate decarboxylase ... and the alpha keto acid pyruvate to meet. ref name pmid8512926 Structure Pyruvate decarboxylase occurs ..., Farrenkopf B, Jordan F title Catalytic centers in the thiamin diphosphate dependent enzyme pyruvate ... of pyruvate decarboxylase width 275 lines 3 Image 1pvd with TPP.jpg Cartoon diagram of pyruvate decarboxylase monomer with TPP attached. Image 1pvd image.jpg Cartoon diagram of pyruvate decarboxylase tetramer. Image PDC chimera cropped r3 c4.jpg Active site of pyruvate decarboxylase with selected ... that participate in conformation changes when interacting with pyruvate substrate. Active site ... are inside a cavity in the core of the enzyme where hydrogen bonding can occur and where the pyruvate ... M, Crout DHG title Pyruvate Decarboxylase A Molecular Modeling Study of Pyruvate Decarboxylation ... 2 sup ion that is present in each active site. To ensure that only pyruvate binds, two Cys 221 more ...   more details



  1. Pyruvate oxidase

    enzyme Name pyruvate oxidase EC number 1.2.3.3 CAS number 9001 96 1 IUBMB EC number 1 2 3 3 GO code 0047112 image width caption In enzymology , a pyruvate oxidase EC number 1.2.3.3 is an enzyme that catalysis catalyzes the chemical reaction pyruvate phosphate O sub 2 sub math rightleftharpoons math acetyl phosphate CO sub 2 sub H sub 2 sub O sub 2 sub The 3 substrate biochemistry substrates of this enzyme are pyruvate , phosphate , and oxygen O sub 2 sub , whereas its 3 product chemistry products are acetyl phosphate , carbon dioxide CO sub 2 sub , and hydrogen peroxide H sub 2 sub O sub 2 sub . This enzyme belongs to the family of oxidoreductase s, specifically those acting on the aldehyde or oxo group of donor with oxygen as acceptor. The systematic name of this enzyme class is pyruvate oxygen 2 oxidoreductase phosphorylating . Other names in common use include pyruvic oxidase , and phosphate dependent pyruvate oxidase . This enzyme participates in pyruvate metabolism . It has 2 cofactor biochemistry cofactors FAD , and Thiamin diphosphate . Structural studies As of late 2007, 12 tertiary structure structures have been solved for this class of enzymes, with Protein Data Bank PDB accession codes PDB link 1POW , PDB link 1POX , PDB link 1V5E , PDB link 1V5F , PDB link 1V5G , PDB link 1Y9D , PDB link 2DJI , PDB link 2EZ4 , PDB link 2EZ8 , PDB link 2EZ9 , PDB link 2EZT , and PDB link 2EZU . References reflist 1 cite journal author Williams FR, Hager LP date 1966 title Crystalline flavin pyruvate oxidase from Escherichia coli. I Isolation and properties of the flavoprotein journal Arch. Biochem. Biophys. volume 116 pages 168&ndash 76 pmid 5336022 doi 10.1016 0003 9861 66 90025 7 issue 1 cite journal author Tittmann K, Wille G, Golbik R, Weidner A, Ghisla S, Hubner G date 2005 title Radical phosphate transfer mechanism for the thiamin diphosphate and FAD dependent pyruvate oxidase from Lactobacillus plantarum Kinetic coupling of intercofactor electron transfer with phosphate ...   more details



  1. Pyruvate synthase

    enzyme Name pyruvate synthase EC number 1.2.7.1 CAS number 9082 51 3 IUBMB EC number 1 2 7 1 GO code 0019164 image width caption In enzymology , a pyruvate synthase EC number 1.2.7.1 is an enzyme that catalysis catalyzes the chemical reaction pyruvate CoA 2 oxidized ferredoxin math rightleftharpoons math acetyl CoA CO sub 2 sub 2 reduced ferredoxin 2 H sup sup The 3 substrate biochemistry substrates of this enzyme are pyruvate , coenzyme A CoA , and oxidized ferredoxin , whereas its 4 product chemistry products are acetyl CoA , carbon dioxide CO sub 2 sub , reduced ferredoxin , and hydrogen ion H sup sup . This enzyme belongs to the family of oxidoreductase s, specifically those acting on the aldehyde or oxo group of donor with an iron sulfur protein as acceptor. The systematic name of this enzyme class is pyruvate ferredoxin 2 oxidoreductase CoA acetylating . Other names in common use include pyruvate oxidoreductase , pyruvate synthetase , pyruvate ferredoxin oxidoreductase , and pyruvic ferredoxin oxidoreductase . This enzyme participates in 4 metabolism metabolic pathways pyruvate metabolism , propanoate metabolism , butanoate metabolism , and reductive carboxylate cycle CO2 fixation . Structural studies As of late 2007, 10 tertiary structure structures have been solved for this class of enzymes, with Protein Data Bank PDB accession codes PDB link 1B0P , PDB link 1KEK , PDB link 2C3M , PDB link 2C3O , PDB link 2C3P , PDB link 2C3U , PDB link 2C3Y , PDB link 2C42 , PDB link 2PDA , and PDB link 2RAA . References reflist 1 cite journal author Evans MC, Buchanan BB date 1965 title Photoreduction of ferredoxin and its use in carbon dioxide fixation by a subcellular system from ... K, Rabinowitz JC date 1971 title Pyruvate ferredoxin oxidoreductase. 3. Purification and properties ... author Uyeda K, Rabinowitz JC date 1971 title Pyruvate ferredoxin oxidoreductase. IV. Studies on the reaction ... Structure and electron transfer mechanism of pyruvate ferredoxin oxidoreductase journal Curr. Opin. Struct ...   more details



  1. Sodium pyruvate

    chembox verifiedrevid 402673135 Name Sodium pyruvate ImageFile Sodium pyruvate.png ImageFileL1 Pyruvate 3D balls.png ImageSizeL1 160px ImageNameL1 Ball and stick model of the pyruvate anion ImageFileR1 Sodium 3D.png ImageSizeR1 80px ImageNameR1 The sodium cation OtherNames ketopropionic acid sodium salt 2 oxopropanoic acid sodium salt Pyruvic acid sodium salt Section1 Chembox Identifiers ChemSpiderID Ref chemspidercite correct chemspider ChemSpiderID 7931 PubChem 8224 UNII Ref fdacite correct FDA UNII POD38AIF08 InChI 1 C3H4O3.Na c1 2 4 3 5 6 h1H3, H,5,6 q 1 p 1 InChIKey DAEPDZWVDSPTHF REWHXWOFAL SMILES Na . O C O C O C StdInChI Ref stdinchicite correct chemspider StdInChI 1S C3H4O3.Na c1 2 4 3 5 6 h1H3, H,5,6 q 1 p 1 StdInChIKey Ref stdinchicite correct chemspider StdInChIKey DAEPDZWVDSPTHF UHFFFAOYSA M CASNo Ref cascite correct CAS CASNo 113 24 6 Section2 Chembox Properties Formula C sub 3 sub H sub 3 sub NaO sub 3 sub MolarMass 110.0 g mol Density Solvent other solvents SolubleOther 100 mg mL MeltingPt BoilingPt Sodium pyruvate is commonly added to cell culture media as an additional source of energy, but may also have protective effects against hydrogen peroxide . This was reported by Giandomenico et al. . ref cite journal author Giandomenico AR, Cerniglia GE, Biaglow JE, Stevens CW, Koch CJ title The importance of sodium pyruvate in assessing damage produced by hydrogen peroxide. journal Free Radic Biol Med volume 23 issue 3 pages 426 34 year 1997 pmid 9214579 doi 10.1016 S0891 5849 97 00113 5 url ref and has been confirmed by several independent groups. References reflist Category Sodium compounds biochem stub ar ...   more details



  1. Pyruvate scale

    The pyruvate scale measures pungency in onions and garlic with units of um gfw. It is named after pyruvic acid , the alpha keto acid co product created in the biochemical pathway that forms the lacrimator, syn Propanethial S oxide . The standard onion has an eight rating, while sweet onions have a two or three rating on the scale. The lower the score or scale the more sweet the onions are rated. Anything less than five is considered a sweet onion. The Vidalia onion variety is considered sweet and must have a score of 5.0 um gfw or less. Fact date February 2007 The Supasweet onion usually grown in Lincolnshire , England registers 1.5 to 2 on the scale. A standard brown onion is usually in the range of 6 7 out of 10. Soil type, rain, and sunlight affect the pungency in onions and garlic and, therefore, their score on the pyruvate scale. External links http www.ams.usda.gov nop indexNet.htm The National Organic Program &mdash by the USDA Failed verification date January 2011 Category Scales Agri stub ...   more details



  1. Pyruvate cycling

    Pyruvate cycling commonly refers to an intracellular loop of spatial movements and chemical transformations involving pyruvate . Spatial movements occur between Mitochondrion mitochondria and cytosol and chemical transformations create various Krebs cycle intermediates. In all variants, pyruvate is imported into the mitochnodrion for processing through part of the Krebb s cycle. In addition to pyruvate, alpha ketoglutarate may also be imported. At various points, the intermediate product is exported to the cytosol for additional transformations and then re imported. Three specific pyruvate cycles ... A pyruvate cycling pathway involving cytosolic NADP dependent isocitrate dehydrogenase regulates ... may exist, such as dissipative or futile pyruvate cycles ref cite journal author Gregory RB, Berry ... of pyruvate cycling to loss of 6 3H& 93 glucose during conversion of glucose to glycogen in hepatocytes ... SR, Aumais A, Joly E, Prentki M title A role for ATP citrate lyase, malic enzyme, and pyruvate ... Heart E, Cline GW, Collis LP, Pongratz RL, Gray JP, Smith PJ title Role for malic enzyme, pyruvate ... in various cell types including islet cells. The pyruvate malate cycle was described in liver and kidney ... of pyruvate cycling in the metabolism of aspartate and glycerate by the perfused rat kidney journal ... doi url ref . section stubs pyruvate malate pyruvate citrate pyruvate isocitrate References reflist ... metabolism, glutaminolysis, and pyruvate cycling but a decreased gamma aminobutyric acid shunt ..., Renstr m E, Mulder H title Anaplerosis via pyruvate carboxylase is required for the fuel induced ... MV, Joseph JW, Ilkayeva O, et al. title Compensatory responses to pyruvate carboxylase suppression in islet ... between pyruvate cycling and glucose stimulated insulin secretion GSIS journal Proceedings of the National ... February pmid 11240373 doi 10.1016 S1357 2725 00 00079 0 cite journal author Thompson SN title Pyruvate ... Pyruvate cycling involving possible oxaloacetate decarboxylase activity journal Biochimica et Biophysica ...   more details



  1. MAP kinase kinase kinase

    MAP kinase kinase kinase or MAP3K or MEKK is a serine threonine specific protein kinase which acts upon MAP kinase kinase . In humans there are at least 19 genes MAP3K1 aka MEKK1 MAP3K2 MAP3K3 aka MAP3K3 MEKK3 MAP3K4 MAP3K5 aka ASK1 MAP3K6 MAP3K7 aka MAP3K7 MEKK7 aka TAK1 MAP3K8 aka TPL2 or Tpl2 MAP3K9 MAP3K10 MAP3K11 aka MAP3K11 MEKK11 aka MLK3 MAP3K12 aka MUK MAP3K13 aka LZK MAP3K14 MAP3K15 MAP3K16 aka TAO1 or TAOK1 MAP3K17 aka TAO2 or TAOK2 RAF1 BRAF ARAF ZAK Perhaps the best characterized MAP3K are the members of the oncogenic RAF family RAF1, BRAF, ARAF , which are effectors of mitogenic ras signaling and which activate the ERK1 2 MAPK3 MAPK1 pathway, through activation of MEK1 MAP2K1 and MEK2 MAP2K2 . MEKK1 activates C Jun N terminal kinases MAPK8 JNK by phosphorylation of its activator SEK1 MAP2K4 . ref cite journal author Yan M, et al. title Activation of stress activated protein kinase by MEKK1 phosphorylation of its activator SEK1 year 1994 journal Nature volume 372 pages 798 800 pmid 7997270 issue 6508 ref MAP3K3 directly regulates the C Jun N terminal kinases MAPK8 JNK and MAPK ERK pathway extracellular signal regulated protein kinase ERK pathways by activating SEK and MEK1 2 respectively it does not regulate the p38 mitogen activated protein kinases p38 pathway. ref cite journal author Ellinger Ziegelbauer H, et al. title Direct activation of the stress activated protein kinase SAPK and extracellular signal regulated protein kinase ERK pathways by an inducible mitogen activated protein Kinase ERK kinase kinase 3 MEKK derivative year 1997 journal J Biol Chem. volume 272 pages 2668 74 doi 10.1074 jbc.272.5.2668 pmid 9006902 issue 5 ref MAP3K7 TAK1 participate in regulation of transcription by transforming growth factor beta TGF beta . ref cite journal author Yamaguchi K, et al. title Identification of a member of the MAPKKK family as a potential mediator of TGF ... MAP Kinase Kinase Kinases EC number 2.7.11.25 References Reflist biochemistry stub MAP kinase activation ...   more details



  1. MAP kinase kinase kinase kinase

    Mitogen activated protein kinase kinase kinase kinase MAP4K is a family of proteins involved in cellular signal transduction . Note This list is probably far from complete, in both the number of molecules and their common alternative names. Original editor has further knowledge but is reluctant to include it because he is still learning. MAP4K1 aka HPK1 MAP4K2 aka GCK MAP4K3 MAP4K4 MAP4K5 Additional Images gallery File Signal transduction v1.png Some signal transduction pathways. MAP4K s not labelled. File MAPKpathway.jpg MAPK pathway. Some or all of the MAP4K s in this image are labelled by other names, such as HPK1 and GCK. gallery See Also Signal transduction MAP kinase MAP kinase kinase MAP kinase kinase kinase biochemistry stub MAP kinase activation Serine threonine specific protein kinases ...   more details




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