chembox verifiedrevid 443553935 ImageFile Ref chemboximage correct ?? ImageFile Cystathionine.png ImageName Skeletal formula ImageFile1 Cystathionine 3D balls.png ImageSize1 230px ImageName1 Ball and stick model IUPACName 2 amino 4 2 amino 2 carboxy ethyl thio butanoic acid OtherNames small L small Cystathionine S 2 R 2 Amino 2 carboxyethyl small L small homocysteine Section1 Chembox Identifiers KEGG Ref keggcite correct kegg KEGG C00542 InChI 1 C7H14N2O4S c8 4 6 10 11 1 2 14 3 5 9 7 12 13 h4 5H,1 3,8 9H2, H,10,11 H,12,13 InChIKey ILRYLPWNYFXEMH UHFFFAOYAH ChEMBL Ref ebicite correct EBI ChEMBL 209241 StdInChI Ref stdinchicite correct chemspider StdInChI 1S C7H14N2O4S c8 4 6 10 11 1 2 14 3 5 9 7 12 13 h4 5H,1 3,8 9H2, H,10,11 H,12,13 StdInChIKey Ref stdinchicite correct chemspider StdInChIKey ILRYLPWNYFXEMH UHFFFAOYSA N CASNo Ref cascite correct CAS CASNo 56 88 2 PubChem 834 ChemSpiderID Ref chemspidercite correct chemspider ChemSpiderID 811 ChEBI Ref ebicite correct EBI ChEBI 17755 SMILES O C O C N CSCCC C O O N MeSHName Cystathionine Section2 Chembox Properties Formula C sub 7 sub H sub 14 sub N sub 2 sub O sub 4 sub S MolarMass 222.263 g mol Appearance Density MeltingPt BoilingPt Solubility Section3 Chembox Hazards MainHazards FlashPt Autoignition Cystathionine is an intermediate in the synthesis of cysteine . It is generated from homocysteine and serine by cystathionine beta synthase . It is cleaved into cysteine and ketobutyrate by cystathionine gamma lyase . An excess in the urine is called cystathioninuria . File Cysteine Biosynthesis.png thumb left 150px Cysteine metabolism. Cystathionine beta synthase catalyzes the upper reaction and cystathionine gamma lyase catalyzes the lower reaction. Amino acid metabolism intermediates biochem stub Category Sulfur amino acids fr Cystathionine ja ... more details
Merge Cystathionine beta lyase date May 2011 Unreferenced date September 2009 Cystathionine beta lyase is an enzyme used in the biosynthesis of methionine . It is not present in humans. It is classified under EC number 4.4.1.8 . See also Cystathionine Carbon sulfur lyases lyase stub Category Enzymes Category Essential amino acids Category Lyases Category Sulfur amino acids ... more details
Refimprove date June 2007 Cystathionine y synthase CGS is an enzyme which plays an important role in regulating the biosynthesis of methionine in bacteria and plants . External links Cite journal author Hacham Y, Avraham T, Amir R title The N terminal region of Arabidopsis cystathionine gamma synthase plays an important regulatory role in methionine metabolism journal Plant Physiol. volume 128 issue 2 pages 454 62 year 2002 month February pmid 11842149 pmc 148908 doi 10.1104 pp.010819 url http www.plantphysiol.org cgi pmidlookup?view long&pmid 11842149 DEFAULTSORT Cystathionine Synthase Category Enzymes Enzyme stub ... more details
enzyme Name cystathionine gamma lyase EC number 4.4.1.1 CAS number 9012 96 8 IUBMB EC number 4 4 1 1 GO code 0004123 image Cysteine Biosynthesis.png width caption Cysteine metabolism. Cystathionase catalyzes the lower reaction. Cystathionine gamma lyase or cystathionase is an enzyme which breaks down cystathionine into cysteine and ketobutyrate . Pyridoxal phosphate is a prosthetic group of this enzyme. Cystathionine gamma lyase also catalyses the following elimination reactions L homoserine to form H sub 2 sub O, NH sub 3 sub and 2 oxobutanoate L cystine , producing thiocysteine, pyruvic acid pyruvate and NH sub 3 sub L cysteine producing pyruvate, NH sub 3 sub and H sub 2 sub S In some bacteria and mammal s, including humans, this enzyme takes part in generating hydrogen sulfide . ref name Wang 2010 cite journal author Wang R title Toxic Gas, Lifesaver journal Scientific American volume issue pages 52 year 2010 month March pmid doi url http www.scientificamerican.com article.cfm?id toxic gas lifesaver ref Examples The following cystathionine gamma lyase is expressed in humans protein Name cystathionase cystathionine gamma lyase caption image width HGNCid 2501 Symbol CTH AltSymbols EntrezGene 1491 OMIM 607657 RefSeq NM 001902 UniProt P32929 PDB ECnumber 4.4.1.1 Chromosome 1 Arm p Band 31.1 LocusSupplementaryData Pathology An deficiency is associated with cystathioninuria . See also Cysteine metabolism External links MeshName Cystathionine gamma lyase References Reflist lyase stub Carbon sulfur lyases Amino acid metabolism enzymes ... more details
Merge Cystathionine beta lyase date May 2011 enzyme Name cystathionine beta lyase EC number 4.4.1.8 CAS number 9055 05 4 IUBMB EC number 4 4 1 8 GO code 0004121 image width caption Orphan date February 2009 In enzymology , a cystathionine beta lyase EC number 4.4.1.8 is an enzyme that catalysis catalyzes the chemical reaction L cystathionine H sub 2 sub O math rightleftharpoons math L homocysteine NH sub 3 sub pyruvate Thus, the two substrate biochemistry substrates of this enzyme are L cystathionine and water H sub 2 sub O , whereas its 3 product chemistry products are L homocysteine , ammonia NH sub 3 sub , and pyruvate . This enzyme belongs to the family of lyase s, specifically the class of carbon sulfur lyases. The systematic name of this enzyme class is L cystathionine L homocysteine lyase deaminating pyruvate forming . Other names in common use include beta cystathionase , cystine lyase , cystathionine L homocysteine lyase deaminating , and L cystathionine L homocysteine lyase deaminating . This enzyme participates in 5 metabolism metabolic pathways methionine metabolism , cysteine metabolism , selenoamino acid metabolism , nitrogen metabolism , and sulfur metabolism . It employs one cofactor biochemistry cofactor , pyridoxal phosphate . Structural studies As of late 2007, 5 tertiary structure structures have been solved for this class of enzymes, with Protein Data Bank PDB accession codes PDB link 1CL1 , PDB link 1CL2 , PDB link 1IBJ , PDB link 2FQ6 , and PDB link 2GQN . References reflist 1 cite journal author Anderson NW and Thompson JF date 1979 title Cystine lyase beta cystathionase from turnip roots journal Phytochemistry journal Phytochemistry volume 18 pages 1953&ndash 1958 doi 10.1016 S0031 9422 00 82710 7 issue 12 cite journal author FLAVIN M, SLAUGHTER C date 1964 title CYSTATHIONINE CLEAVAGE ENZYMES OF NEUROSPORA journal J. Biol. Chem. volume 239 pages 2212&ndash 9 pmid 14209950 Category EC 4.4.1 Category Pyridoxal phosphate enzymes Category Enzymes ... more details
Orphan date July 2011 enzyme Name cystathionine gamma synthase EC number 2.5.1.48 CAS number 9030 70 0 IUBMB EC number 2 5 1 48 GO code 0003962 image width caption In enzymology , a cystathionine gamma synthase EC number 2.5.1.48 is an enzyme that catalysis catalyzes the chemical reaction O sub 4 sub succinyl L homoserine L cysteine math rightleftharpoons math L cystathionine succinate Thus, the two substrate biochemistry substrates of this enzyme are O4 succinyl L homoserine and L cysteine , whereas its two product chemistry products are L cystathionine and succinate . This enzyme belongs to the family of transferase s, specifically those transferring aryl or alkyl groups other than methyl groups. The systematic name of this enzyme class is O4 succinyl L homoserine L cysteine S 3 amino 3 carboxypropyl transferase . Other names in common use include O succinyl L homoserine succinate lyase adding cysteine , O succinylhomoserine thiol lyase , homoserine O transsuccinylase , O succinylhomoserine synthase , O succinylhomoserine synthetase , cystathionine synthase , cystathionine synthetase , homoserine transsuccinylase , 4 O succinyl L homoserine L cysteine , and S 3 amino 3 carboxypropyl transferase . This enzyme participates in 4 metabolism metabolic pathways methionine metabolism , cysteine metabolism , selenoamino acid metabolism , and sulfur metabolism . It employs one cofactor biochemistry cofactor , pyridoxal phosphate . References reflist 1 cite journal author Flavin M, Slaughter C year 1967 title Enzymatic synthesis of homocysteine or methionine directly from O succinyl homoserine journal Biochim. Biophys. Acta. volume 132 pages 400&ndash 5 pmid 5340123 issue 2 cite journal author Kaplan MM, Flavin M year 1966 title Cystathionine gamma synthetase of Salmonella. Catalytic ... MC, Messerschmidt A year 1998 title Crystal structure of Escherichia coli cystathionine gamma synthase ... 2 title Cystathionine gamma synthase from Arabidopsis thaliana purification and biochemical characterization ... more details
PBB geneid 875 Cystathionine synthase , also known as CBS , is an enzyme EC number 4.2.1.22 that in humans ... homocysteine to cystathionine ref cite web title Entrez Gene CBS cystathionine beta synthase url ... L serine homocysteine L homocysteine math rightleftharpoons math cystathionine L cystathionine ... Regulation of human cystathionine beta synthase by S adenosyl L methionine evidence for two catalytically ... homocysteine L cystathionine forming . Other names in common use include beta thionase, cysteine synthase ... of the CBS enzyme. ref name pmid11483494 The human enzyme cystathionine synthase is a tetrameric ... CG title Redox regulation and reaction mechanism of human cystathionine beta synthase a PLP dependent ... cystathionine beta synthase journal Journal of Inorganic Biochemistry volume 100 issue 12 pages 1988 ... proteins. ref name Ruma CBS has a C terminal inhibitory domain. The C terminal domain of cystathionine ... R title Human cystathionine beta synthase is a target for sumoylation journal Biochemistry volume ... activity enzyme Name cystathionine beta synthase EC number 4.2.1.22 CAS number 9023 99 8 IUBMB ... Cysteine metabolism. Cystathionine beta synthase catalyzes the upper reaction and cystathionine gamma lyase catalyzes the lower reaction. Transsulfuration, catalyzed by CBS, converts homocysteine to cystathionine ... and molecular characterization of cystathionine beta synthase and serine acetyltransferase from ... at C generate the external aldimine of cystathionine . A final transaldimination reaction releases the final product, cystathionine. ref name Ruma The final product, L cystathionine can .... ref name Jhee cite journal author Jhee KH, Kruger WD title The role of cystathionine beta synthase ... by homocysteine binding and release of cystathionine. In contrast, the steady state enzyme ... involving CBS is the condensation of cysteine with homocysteine to form cystathionine and hydrogen ... producing activity of cystathionine beta synthase journal The Journal of Biological Chemistry volume ... more details
Infobox Disease Name Cystathioninuria Image L Cystathionine.svg Caption Cystathionine DiseasesDB 29671 ICD10 ICD10 E 72 1 e 70 ICD9 ICD9 270.4 ICDO OMIM 219500 MedlinePlus eMedicineSubj eMedicineTopic MeshID Cystathioninuria , also called cystathionase deficiency , is an autosomal recessive ref name omim OMIM 219500 ref metabolic disorder that results in an excess of cystathionine in the urine . It is associated with a congenital dysfunction of the enzyme cystathionase , or acquired deficiency of vitamin B6 vitamin B sub 6 sub which is essential for the function of this enzyme. The latter is usually related to an overall deficiency of all the B complex vitamins. Genetics Image Autorecessive.jpg thumb left Cystathioninuria has an autosomal recessive pattern of heredity inheritance . Cystathioninuria is inherited in an autosomal recessive manner. ref name omim This means the defective gene responsible for the disorder is located on an autosome , and two copies of the defective gene one inherited from each parent are required in order to be born with the disorder. The parents of an individual with an autosomal recessive disorder both genetic carrier carry one copy of the defective gene, but usually do not experience any signs or symptoms of the disorder. Pathophysiology File Cysteine Biosynthesis.png thumb right 150px Cysteine metabolism. Cystathionine gamma lyase cystathionase catalyzes the lower reaction. References reflist Amino acid metabolic pathology Category Amino acid metabolism disorders Category Autosomal recessive disorders genetic disorder stub ... more details
The transsulfuration pathway is a metabolic pathway involving the interconversion of cysteine and homocysteine , through the intermediate cystathionine . In eukarotes, such as humans, the transsulfuration pathway is critical for creating cysteine from the essential amino acid methionine. Methionine is first converted to homocysteine by demethylation, which is then converted to the amino acid cysteine via the transsulfuration pathway. While methionine is considered an essential amino acid, cysteine becomes an essential amino acid when the transsulfuration pathway is defective. In organisms that synthesize cysteine in sulfur assimilation such as bacteria and yeast, the transsulfuration pathway works in reverse. The production of homocysteine through transsulfuration allows the conversion of this intermediate to methionine , through a methylation reaction carried out by 5 Methyltetrahydrofolate homocysteine methyltransferase methionine synthase . biochemistry stub Category Nitrogen metabolism Category Sulfur metabolism Category Metabolic pathways vi Chu tr nh chuy n sunfua h a ... more details
NIH UW entry on Homocystinuria Caused by Cystathionine Beta Synthase Deficiency http docs.google.com ... Paper and discussion on Homocystinuria due to Cystathionine Beta Synthase deficiency References ... more details
to O succinyl homoserine , which then reacts with cysteine to produce cystathionine , which is cleaved ... methionine. Both cystathionine synthase and cystathionine lyase require Pyridoxal phosphate ... homoserine O transsuccinylase cystathionine synthase cystathionine lyase methionine synthase ... than methionine synthase. Conversion to cysteine Homocysteine can be converted to cysteine. 5 Cystathionine synthase a PLP dependent enzyme combines homocysteine and serine to produce cystathionine . Instead of degrading cystathionine via cystathionine lyase , as in the biosynthetic pathway, cystathionine is broken down to cysteine and ketobutyrate via 6 cystathionine lyase . 7 The enzyme ... more details
, cystathionine, cysteine Preparation and properties. In Sulfur Amino Acids Biochemical and Clinical ... contributions of cystathionine beta synthase and gamma cystathionase to H2S biogenesis via alternative ... more details
Unreferenced date December 2009 Cysteine metabolism refers to the biological pathways that consume or create cysteine . The pathways of different amino acids and other metabolites interweave and overlap to creating complex systems. Human cysteine metabolism In human cysteine metabolism, L cysteine is consumed in several ways as shown below. L cysteine is also consumed in methionine and glutathione metabolism as well as pantothenate CoA biosynthesis. class wikitable style text align center L cysteine consumption pathways enzyme product cysteine dioxygenase 3 sulfino L alanine or cysteine sulfinic acid amino acid racemase D cysteine cysteine lyase L cysteate cysteine tRNA ligase L cysteinyl tRNA sup Cys sup cystine reductase L cystine cysteine transaminase 3 mercapto pyruvate L cysteine is the product of several processes as well. In addition to the reactions below, L cysteine is also a product of glycine , serine , and threonine metabolism. class wikitable style text align center L cysteine production pathways starting chemical enzyme O acetyl L serine cysteine synthase L cystine glutathione cystine transhydrogenase pyruvate cystathionine lyase 3 mercapto pyruvate cysteine transaminase See also D cysteine desulfhydrase Sulphur metabolism DEFAULTSORT Cysteine Metabolism Category Sulfur metabolism Category Sulfur amino acids ja ... more details
enzyme Name homoserine O succinyltransferase EC number 2.3.1.46 CAS number 62213 51 8 IUBMB EC number 2 3 1 46 GO code 0008899 image width caption In enzymology , a homoserine O succinyltransferase EC number 2.3.1.46 is an enzyme that catalysis catalyzes the chemical reaction succinyl CoA L homoserine math rightleftharpoons math CoA O succinyl L homoserine Thus, the two substrate biochemistry substrates of this enzyme are succinyl CoA and L homoserine , whereas its two product chemistry products are coenzyme A CoA and O succinyl L homoserine . This enzyme belongs to the family of transferase s, specifically those acyltransferase s transferring groups other than aminoacyl groups. The systematic name of this enzyme class is succinyl CoA L homoserine O succinyltransferase . Other names in common use include homoserine O transsuccinylase , and homoserine succinyltransferase . This enzyme participates in methionine metabolism and sulfur metabolism . Structural studies As of late 2007, two tertiary structure structures have been solved for this class of enzymes, with Protein Data Bank PDB accession codes PDB link 2GHR and PDB link 2H2W . References reflist 1 cite journal author Rowbury RJ and Woods DD date 1964 title O Succinylhomoserine as an intermediate in the synthesis of cystathionine by Escherichia coli journal J. Gen. Microbiol. volume 36 pages 341&ndash 358 pmid 14217349 transferase stub Category EC 2.3.1 Category Enzymes of known structure it Omoserina O succiniltransferasi ... more details
wiktionarypar CSE CSE may refer to Computing Collaborative search engine Common subexpression elimination , a compiler optimization technique Comparison Shopping Engine Computational Science and Engineering , the science and engineering of computation, usually associated with High Performance Computing Computer Systems Engineering , a Bachelor of Engineering course Google Custom Search Engine Custom Search Engine , a Google service to create personalized search engines Education Examinations Certificate of Secondary Education , a UK examination Civil Services Examination , a competitive examination in India Schools Coll ge du Saint Esprit , a Catholic, boys only, secondary school based in Quatre Bornes, Mauritius. College of Saint Elizabeth College of Science and Engineering &ndash the new name, as of July 2010, of the University of Minnesota s University of Minnesota Institute of Technology Institute of Technology University programs Chalmers School of Entrepreneurship , a master s program at Chalmers University of Technology and University of Gothenburg Field Control Systems Engineering , a broad field of engineering Finance Calcutta Stock Exchange Casablanca Stock Exchange Colombo Stock Exchange , a Sri Lankan stock exchange Cyprus Stock Exchange Medical Combined spinal and epidural anaesthesia , an anaesthetic technique Cystathionine gamma lyase , an enzyme Organisations Companies Compa a Sevillana de Electricidad Governmental Communications Security Establishment Canada , a Canadian intelligence agency Non governmental Centre for Science and Environment , an Indian non governmental organization Corpus Speculorum Etruscorum , a project to publish all existing Etruscan mirrors Council of Science Editors the CSE citation style is used in some natural sciences works Creation Science Evangelism , a Christian apologetics ministry of Kent Hovind Sporting clubs Clube Sociedade Esportiva , a Brazilian football soccer club Professions Cisco Sales Expert, a designation b ... more details
Marfanoid or Marfanoid habitus is a term used to describe a constellation of findings including long limbs, arachnodactyly and hyperlaxity . Associated conditions include Marfan syndrome the source of the name Multiple endocrine neoplasia type 2B ref name pmid15586612 cite journal author Prabhu M, Khouzam RN, Insel J title Multiple endocrine neoplasia type 2 syndrome presenting with bowel obstruction caused by intestinal neuroma case report journal South. Med. J. volume 97 issue 11 pages 1130 2 year 2004 month November pmid 15586612 doi 10.1097 01.SMJ.0000140873.29381.12 url http meta.wkhealth.com pt pt core template journal lwwgateway media landingpage.htm?issn 0038 4348&volume 97&issue 11&spage 1130 ref ref name pmid17963006 cite journal author Wray CJ, Rich TA, Waguespack SG, Lee JE, Perrier ND, Evans DB title Failure to recognize multiple endocrine neoplasia 2B more common than we think? journal Ann. Surg. Oncol. volume 15 issue 1 pages 293 301 year 2008 month January pmid 17963006 doi 10.1245 s10434 007 9665 4 ref Homocystinuria ref Cite journal last1 Pagon first1 RA. last2 Bird first2 TC. last3 Dolan first3 CR. last4 Stephens first4 K. last5 Picker first5 JD. last6 Levy first6 HL. title Homocystinuria Caused by Cystathionine Beta Synthase Deficiency journal volume issue pages month year 1993 doi pmid 20301697 ref References reflist Category Congenital disorders medicine stub ... more details
DISPLAYTITLE alpha Ketobutyric acid chembox verifiedrevid 443659318 Name Ketobutyric acid ImageFile Alpha ketobutyric acid.svg ImageSize ImageFile1 Alpha Ketobutyric acid.png ImageSize IUPACName 2 oxobutanoic acid OtherNames Section1 Chembox Identifiers KEGG Ref keggcite correct kegg KEGG C00109 InChI 1 C4H6O3 c1 2 3 5 4 6 7 h2H2,1H3, H,6,7 InChIKey TYEYBOSBBBHJIV UHFFFAOYAT ChEMBL Ref ebicite correct EBI ChEMBL 171246 StdInChI Ref stdinchicite correct chemspider StdInChI 1S C4H6O3 c1 2 3 5 4 6 7 h2H2,1H3, H,6,7 StdInChIKey Ref stdinchicite correct chemspider StdInChIKey TYEYBOSBBBHJIV UHFFFAOYSA N CASNo Ref cascite correct CAS CASNo 600 18 0 PubChem 58 ChemSpiderID Ref chemspidercite correct chemspider ChemSpiderID 57 ChEBI Ref ebicite correct EBI ChEBI 30831 DrugBank Ref drugbankcite correct drugbank DrugBank DB04553 SMILES O C C O O CC MeSHName Alpha ketobutyric acid Section2 Chembox Properties Formula C sub 4 sub H sub 6 sub O sub 3 sub MolarMass 102.089 g mol Appearance Density MeltingPt BoilingPt Solubility Section3 Chembox Hazards MainHazards FlashPt Autoignition Ketobutyric acid is a product of the lysis of cystathionine . It is also one of the degradation products of threonine , produced by the catabolism of the amino acid by threonine dehydratase . It is also produced by the degradation of homocysteine and the metabolism of methionine . Ketobutyric acid is transported into the mitochondrial matrix, where it is converted to propionyl CoA by Branched Chain Alpha Keto Acid Dehydrogenase Complex . Further mitochondrial reactions produce succinyl CoA . This is first through the enzyme mitochondria Propionyl CoA Carboxylase with Biotin as a cofactor to produce S methylmalonyl CoA. This is subsequently converted to R methylmalonyl CoA by mitochondrial Methylmalonyl CoA epimerase . Finally, mitochondrial Methylmalonyl CoA Mutase with cofactor Adenosylcoalbamin produces succinyl CoA which enters the citric acid cycle . ref http pathman.smpdb.ca pathways SMP00 ... more details
. Cystathionine beta synthase Cystathionine synthase catalyses the condensation of homocysteine and serine to give cystathionine . This reaction uses pyridoxine vitamin B sub 6 sub as a cofactor. Cystathionine beta lyase Cystathionine lyase then converts this double amino acid to cysteine ... more details
, a compound that SDH combines with Serine to create cystathionine , also noncompetitively inhibits ... levels of homocysteine. L Serine dehydratase condenses homocysteine with serine to form cystathionine ... of human cystathionine synthaseEvidence for the identity of human l serine dehydratase and cystathionine ... more details
Metabolic File Cysteine Biosynthesis.png thumb 150px Cysteine synthesis from serine. Cystathionine beta synthase catalyzes the upper reaction and cystathionine gamma lyase catalyzes the lower reaction ... more details
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