enzyme Name phosphoribosylaminoimidazolecarboxylase EC number 4.1.1.21 CAS number 9032 04 6 IUBMB EC number 4 1 1 21 GO code 0004638 image width caption protein Name phosphoribosylaminoimidazolecarboxylase, phosphoribosylaminoimidazole succinocarboxamide synthetase caption image width HGNCid 8587 Symbol PAICS AltSymbols PAIS EntrezGene 10606 OMIM 172439 RefSeq NM 006452 UniProt P22234 PDB ECnumber 4.1.1.21 Chromosome 4 Arm p Band ter LocusSupplementaryData q21 Phosphoribosylaminoimidazolecarboxylase or AIR carboxylase is an enzyme involved in nucleotide biosynthesis and in particular in purine biosynthesis . It catalyzes the conversion of 5 phosphoribosyl 5 aminoimidazole AIR into 5 Phosphoribosyl 4 carboxy 5 aminoimidazole 5 phosphoribosyl 4 carboxy 5 aminoimidazole CAIR as described in the reaction 5 aminoimidazole ribonucleotide CO sub 2 sub math rightleftharpoons math 5 phosphoribosyl 4 carboxy 5 aminoimidazole 2 H sup sup In plants and fungi Phosphoribosylaminoimidazolecarboxylase is a fusion protein in plant s and fungi , but consists of two non interacting proteins in bacteria, PurK and PurE. The crystal structure of PurE indicates a unique quaternary structure that confirms the octameric nature of the enzyme. ref name PUB00016906 cite journal doi 10.1016 S0969 2126 00 80029 5 author Ealick SE, Stubbe J, Kappock TJ, Mathews II title Crystal structure of Escherichia coli PurE, an unusual mutase in the purine biosynthetic pathway journal Structure volume 7 issue 11 pages 1395 1406 year 1999 pmid 10574791 ref In Escherichia coli In the bacteria bacterium Escherichia coli the reaction is catalyzed in two steps carried out by two separate enzymes, PurK and PurE. PurK, N5 carboxyaminoimidazole ribonucleotide synthetase N 5 carboxyaminoimidazole ribonucleotide synthetase , catalyzes the conversion of 5 aminoimidazole ribonucleotide AIR , Adenosine triphosphate ATP ... links MeshName phosphoribosylaminoimidazolecarboxylase http bioch.szote.u szeged.hu astrojan protein ... more details
enzyme Name Phosphoribosylaminoimidazole synthetase EC number 2.1.2.2 CAS number 9032 02 4 IUBMB EC number 2 1 2 2 GO code 0004644 image width caption In the field of enzymology , phosphoribosylaminoimidazole synthetase is an enzyme that catalyzes the chemical reaction 10 formyltetrahydrofolate N1 5 phospho D ribosyl glycinamide math rightleftharpoons math tetrahydrofolic acid tetrahydrofolate N2 formyl N1 5 phospho D ribosyl glycinamide This enzyme is required for the de novo biosynthesis of purine s. References refbegin cite journal author Hartman SC, Buchanan JM title Biosynthesis of the purines. XXVI. The identification of the formyl donors of the transformylation reactions journal J. Biol. Chem. volume 234 issue 7 pages 1812 6 year 1959 month July pmid 13672969 doi url issn cite journal author Smith GK, Benkovic PA, Benkovic SJ title L 10 Formyltetrahydrofolate is the cofactor for glycinamide ribonucleotide transformylase from chicken liver journal Biochemistry volume 20 issue 14 pages 4034 6 year 1981 month July pmid 7284307 doi 10.1021 bi00517a013 url issn cite journal author Warren L, Buchanan JM title Biosynthesis of the purines. XIX. 2 Amino N ribosylacetamide 5 phosphate glycinamide ribotide transformylase journal J. Biol. Chem. volume 229 issue 2 pages 613 26 year 1957 month December pmid 13502326 doi url issn refend transferase stub Category EC 2.1.2 ja ... more details
enzyme Name biotin carboxylase EC number 6.3.4.14 CAS number 9075 71 2 IUBMB EC number 6 3 4 14 GO code 0004075 image width caption Infobox protein family Symbol Biotin carb C Name Biotin carboxylase C terminal domain image PDB 1w96 EBI.jpg width caption crystal structure of biotin carboxylase domain of acetyl coenzyme a carboxylase from saccharomyces cerevisiae in complex with soraphen a Pfam PF02785 Pfam clan InterPro IPR005482 SMART PROSITE MEROPS SCOP 1dv1 TCDB OPM family OPM protein CAZy CDD In enzymology , a biotin carboxylase EC number 6.3.4.14 is an enzyme that catalysis catalyzes the chemical reaction ATP biotin carboxyl carrier protein CO sub 2 sub math rightleftharpoons math ADP phosphate carboxybiotin carboxyl carrier protein The 3 substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP , biotin carboxyl carrier protein , and carbon dioxide CO sub 2 sub , whereas its 3 product chemistry products are adenosine diphosphate ADP , phosphate , and carboxybiotin carboxyl carrier protein . This enzyme belongs to the family of ligase s, specifically those forming generic carbon nitrogen bonds. The systematic name of this enzyme class is biotin carboxyl carrier protein carbon dioxide ligase ADP forming . This enzyme is also called biotin carboxylase component of Acetyl CoA carboxylase acetyl CoA carboxylase . This enzyme participates in fatty acid biosynthesis . A C terminal conserved domain within this enzyme contains most of the active site residues. ref name pmid7915138 Cite pmid 7915138 ref Structural studies As of 2007 alt 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 1BNC , PDB link 1DV1 , PDB link 1DV2 , PDB link 2GPS , and PDB link 2GPW . References reflist 1 Further reading reflist 1 cite journal author Dimroth P, Guchhait RB, Stoll E ... enzyme of acetyl CoA carboxylase journal Proc. Natl. Acad. Sci. U. S. A. volume 67 pages 1353 ... more details
enzyme Name phosphoenolpyruvate carboxylase EC number 4.1.1.31 CAS number 9067 77 0 IUBMB EC number 4 1 1 31 GO code 0008964 image width caption Pfam box Symbol PEPcase Name Phosphoenolpyruvate carboxylase image width caption Pfam PF00311 InterPro IPR001449 SMART Prosite PDOC00330 SCOP 1fiy TCDB OPM family OPM protein PDB Phosphoenolpyruvate carboxylase also known as PEP carboxylase , PEPCase , or PEPC http www.genome.jp dbget bin www bget?enzyme 4.1.1.31 EC 4.1.1.31 is an enzyme in the family of carboxy lyases that catalyzes the addition of bicarbonate to phosphoenolpyruvate PEP to form the four carbon compound oxaloacetate PEP HCO sub 3 sub sup sup oxaloacetate Pi This reaction is used for carbon fixation in so called Crassulacean acid metabolism CAM and C4 carbon fixation C4 plants where it plays a key role in photosynthesis . The enzyme is also found in some bacteria, but not in animals or fungi. ref http www.ncbi.nlm.nih.gov sutils blink.cgi?pid 79319816 NCBI BLINK entry for Arabidopsis PEP carboxylase ref PEP carboxylase in photosynthesis After conversion of carbon dioxide CO sub 2 sub to bicarbonate by carbonic anhydrase , PEP carboxylase assimilates the available bicarbonate into a four carbon compound oxaloacetate, which is further converted to malate that can be stored or shuttled between plant cells. This allows for a separation of initial carbon fixation by contact with air and secondary carbon fixation into sugars by RuBisCO during the light independent reaction s of photosynthesis. In succulent CAM plants adapted for growth in very dry conditions, PEP carboxylase ... plants, for example maize, PEP carboxylase fixes bicarbonate in the mesophyll cells of the leaf ... . As such, it is a wasteful reaction for the plant. By comparison, C4 carbon fixation via PEP carboxylase ... Carboxylase http web.archive.org web 20050509023712 http pfam.wustl.edu cgi bin getdesc ... stub de Phosphoenolpyruvatcarboxylase fr Phospho nolpyruvate carboxylase ... more details
enzyme Name acetone carboxylase EC number 6.4.1.6 CAS number IUBMB EC number 6 4 1 6 GO code 0018710 image width caption In enzymology , an acetone carboxylase EC number 6.4.1.6 is an enzyme that catalysis catalyzes the chemical reaction acetone CO sub 2 sub ATP 2 H sub 2 sub O math rightleftharpoons math acetoacetate AMP 2 phosphate The 4 substrate biochemistry substrates of this enzyme are acetone , carbon dioxide CO sub 2 sub , adenosine triphosphate ATP , and water H sub 2 sub O , whereas its 3 product chemistry products are acetoacetate , adenosine monophosphate AMP , and phosphate . This enzyme belongs to the family of ligase s, specifically those forming carbon carbon bonds. The systematic name of this enzyme class is acetone carbon dioxide ligase AMP forming . References reflist 1 cite journal author Sluis MK, Ensign SA date 1997 title Purification and characterization of acetone carboxylase from Xanthobacter strain Py2 journal Proc. Natl. Acad. Sci. U. S. A. volume 94 pages 8456&ndash 61 pmid 9237998 doi 10.1073 pnas.94.16.8456 issue 16 pmc 22955 ligase stub Category EC 6.4.1 Category Enzymes of unknown structure ... more details
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 from Rhizobium Rhizobium etli biotin carboxylase domain blue allosteric linking domain green biotin binding domain red ... of pyruvate carboxylase journal Biochem. J. volume 413 issue 3 pages 369 87 year 2008 month ... PDB2 1rqe PDB2 1s3h PDB2 1sr9 PDB2 1u5j Protein Name Pyruvate carboxylase image caption Symbol PC ... 608786 EntrezGene 5091 RefSeq NM 000920 UniProt P11498 PDB Pyruvate carboxylase PC is an enzyme of the ligase ... . ref name pmid18613815 Pyruvate carboxylase was first discovered in 1959 at Western Reserve University ... appearance of pyruvate carboxylase from chicken liver journal J. Biol. Chem. volume 254 issue 5 pages ... Puig A, Wallace JC title Anaplerotic roles of pyruvate carboxylase in mammalian tissues journal Cell ... Y, Wallace JC title Molecular cloning and domain structure of chicken pyruvate carboxylase journal Biochem ... spliced pyruvate carboxylase mRNAs with divergent 5 untranslated regions which are expressed in a tissue ... author Kondo S, Nakajima Y, Sugio S, Yong Biao J, Sueda S, Kondo H title Structure of the biotin carboxylase subunit of pyruvate carboxylase from Aquifex aeolicus at 2.2 A resolution journal Acta Crystallogr ... G, Gil D, Valle M, Tong L title A symmetrical tetramer for S. aureus pyruvate carboxylase in complex ..., Rayment I title Domain architecture of pyruvate carboxylase, a biotin dependent multifunctional enzyme ... gallery title Pyruvate Carboxylase Symmetry Comparison width 200 height 150 lines 2 Pyruvate Carboxylase 2QF7, sswilson7.png Rhizobium Rhizobium etli Rhizobium Rhizobium etli Pyruvate carboxylase 3HO8, sswilson7.png Staphylococcus aureus Staphylococcus aureus Pyruvate carboxylase uses a covalently ... Reaction mechanism File Mechanism of Pyruvate Carboxylase, 5 15 2010, sswilson7.png thumb ... more details
enzyme Name urea carboxylase EC number 6.3.4.6 CAS number 9058 98 4 IUBMB EC number 6 3 4 6 GO code 0004847 image width caption In enzymology , an urea carboxylase EC number 6.3.4.6 is an enzyme that catalysis catalyzes the chemical reaction ATP urea HCO sub 3 sub math rightleftharpoons math ADP phosphate urea 1 carboxylate The 3 substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP , urea , and HCO3 , whereas its 3 product chemistry products are adenosine diphosphate ADP , phosphate , and urea 1 carboxylate allophanate . This enzyme belongs to the family of ligase s, specifically those forming generic carbon nitrogen bonds. The systematic name of this enzyme class is urea carbon dioxide ligase ADP forming . Other names in common use include urease ATP hydrolysing , urea carboxylase hydrolysing , ATP urea amidolyase , urea amidolyase , UALase , and UCA . This enzyme participates in urea cycle and metabolism of amino groups . It employs one cofactor biochemistry cofactor , biotin . See also Allophanate hydrolase References reflist 1 cite journal author Roon RJ and Levenberg B date 1970 title ATP Urea amidolyase ADP Candida utilis journal Methods Enzymol. volume 17A pages 317&ndash 324 cite journal author Roon RJ, Levenberg B date 1972 title Urea amidolyase. I. Properties of the enzyme from Candida utilis journal J. Biol. Chem. volume 247 pages 4107&ndash 13 pmid 4556303 issue 13 cite journal author Sumrada RA, Cooper TG date 1982 title Urea carboxylase and allophanate hydrolase are components of a multifunctional protein in yeast journal J. Biol. Chem. volume 257 pages 9119&ndash 27 pmid 6124544 issue 15 cite journal author Kanamori T, Kanou N, Atomi H, Imanaka T date 2004 title Enzymatic characterization of a prokaryotic urea carboxylase journal J. Bacteriol. volume 186 pages 2532&ndash 9 pmid 15090492 doi 10.1128 JB.186.9.2532 2539.2004 issue 9 pmc 387783 ligase stub Category EC 6.3.4 Category Biotin enzymes Category Enzymes of unknown structure ... more details
enzyme Name 2 oxoglutarate carboxylase EC number 6.4.1.7 CAS number IUBMB EC number 6 4 1 7 GO code image width caption orphan date December 2008 In enzymology , a 2 oxoglutarate carboxylase EC number 6.4.1.7 is an enzyme that catalysis catalyzes the chemical reaction ATP 2 oxoglutarate HCO sub 3 sub math rightleftharpoons math ADP phosphate oxalosuccinate The 3 substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP , 2 oxoglutarate , and HCO3 , whereas its 3 product chemistry products are adenosine diphosphate ADP , phosphate , and oxalosuccinate . This enzyme belongs to the family of ligase s, specifically those forming carbon carbon bonds. The systematic name of this enzyme class is . Other names in common use include oxalosuccinate synthetase , carboxylating factor for ICDH incorrect , CFI , and OGC . References reflist 1 cite journal author Aoshima M, Ishii M, Igarashi Y date 2004 title A novel biotin protein required for reductive carboxylation of 2 oxoglutarate by isocitrate dehydrogenase in Hydrogenobacter thermophilus TK 6 journal Mol. Microbiol. volume 51 pages 791&ndash 8 pmid 14731279 doi 10.1046 j.1365 2958.2003.03863.x issue 3 cite journal author Aoshima M, Igarashi Y date 2006 title A novel oxalosuccinate forming enzyme involved in the reductive carboxylation of 2 oxoglutarate in Hydrogenobacter thermophilus TK 6 journal Mol. Microbiol. volume 62 pages 748&ndash 59 pmid 17076668 doi 10.1111 j.1365 2958.2006.05399.x issue 3 Category EC 6.4.1 Category Enzymes of unknown structure ligase stub ... more details
enzyme Name acetyl CoA carboxylase phosphatase EC number 3.1.3.44 CAS number 77000 10 3 IUBMB EC number 3 1 3 44 GO code 0050406 image width caption In enzymology , a acetyl CoA carboxylase phosphatase EC number 3.1.3.44 is an enzyme that catalysis catalyzes the chemical reaction acetyl CoA carboxylase phosphate H sub 2 sub O math rightleftharpoons math acetyl CoA carboxylase phosphate Thus, the two substrate biochemistry substrates of this enzyme are acetyl CoA carboxylase phosphate and water H sub 2 sub O , whereas its two product chemistry products are acetyl CoA carboxylase 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 acetyl CoA carbon dioxide ligase ADP forming phosphate phosphohydrolase . References reflist 1 cite journal author Krakower GR and Kim K H date 1980 title Purification and properties of acetyl CoA carboxylase phosphatase journal J. Biol. Chem. volume 256 issue 5 pages 2408&ndash 2413 pmid 6257718 DEFAULTSORT Acetyl CoA carboxylase phosphatase Category EC 3.1.3 Category Enzymes of unknown structure hydrolase stub ... more details
enzyme Name acetyl CoA carboxylase kinase EC number 2.7.11.27 CAS number 77000 06 7 IUBMB EC number 2 7 11 27 GO code 0050405 image width caption In enzymology , a acetyl CoA carboxylase kinase EC number 2.7.11.27 is an enzyme that catalysis catalyzes the chemical reaction ATP acetyl CoA carboxylase math rightleftharpoons math ADP acetyl CoA carboxylase phosphate Thus, the two substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP and acetyl CoA carboxylase , whereas its two product chemistry products are adenosine diphosphate ADP and acetyl CoA carboxylase phosphate. This enzyme belongs to the family of transferase s, specifically those transferring a phosphate group to the sidechain oxygen atom of serine or threonine residues in protein s protein serine threonine kinase s . The systematic name of this enzyme class is ATP acetyl CoA carboxylase phosphotransferase . Other names in common use include acetyl coenzyme A carboxylase kinase phosphorylating , acetyl CoA carboxylase bound kinase , acetyl CoA carboxylase kinase , acetyl CoA carboxylase kinase cAMP independent , acetyl CoA carboxylase kinase 2 , acetyl CoA carboxylase kinase 2 , acetyl CoA carboxylase kinase 3 AMP activated , acetyl coenzyme A carboxylase kinase , ACK2 , ACK3 , AMPK , I peptide kinase , and STK5 . References reflist 1 cite journal author Jamil H, Madsen NB date 1987 title Phosphorylation state of acetyl coenzyme A carboxylase. I. Linear inverse relationship to activity ratios at different ... phosphorylates and inactivates acetyl CoA carboxylase journal J. Biol. Chem. volume 257 pages 1897 ... of three cyclic AMP independent acetyl CoA carboxylase kinases from lactating rat mammary gland and characterization ... acetyl CoA carboxylase kinase from rat liver journal J. Biol. Chem. volume 269 pages 6859&ndash ... CoA carboxylase journal FEBS. Lett. volume 422 pages 43&ndash 6 pmid 9475166 doi 10.1016 S0014 5793 97 01597 4 issue 1 DEFAULTSORT Acetyl CoA carboxylase kinase Category EC 2.7.11 Category Enzymes of unknown ... more details
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 of these substances can damage the body s organs and tissues, particularly in the nervous system. Pyruvate carboxylase deficiency is a rare condition, with an estimated incidence of 1 in 250,000 births worldwide. This disorder appears ... carboxylase deficiency, which are distinguished by the severity of their signs and symptoms .... Children with pyruvate carboxylase deficiency type A typically survive only into early childhood. Type B Pyruvate carboxylase deficiency type B has life threatening signs and symptoms that become apparent ... for less than 3 months after birth. Genetics Image autorecessive.svg thumb right Pyruvate carboxylase ... gene cause pyruvate carboxylase deficiency. The PC gene provides instructions for making an enzyme called pyruvate carboxylase . This enzyme is active in mitochondria , which are the energy producing ... of glucose , a simple sugar that is the body s main energy source. Pyruvate carboxylase also plays ... of pyruvate carboxylase in cells or disrupt the enzyme s activity. The missing or altered enzyme cannot ... in mitochondria. Additionally, a loss of pyruvate carboxylase allows potentially toxic compounds ... of pyruvate carboxylase function in the nervous system, particularly the role of the enzyme ... carboxylase deficiency. This condition is inherited in an autosomal recessive pattern, which ...?book gene&part pdc GeneReview NCBI NIH UW entry on Pyruvate Carboxylase Deficiency See also NLM ... Pyruvate Carboxylase Deficiency Category Autosomal recessive disorders Category Inborn errors of carbohydrate ... more details
Infobox Disease Name PAGENAME Image Caption DiseasesDB ICD10 ICD9 ICDO OMIM MedlinePlus eMedicineSubj eMedicineTopic MeshID D009100 Multiple carboxylase deficiency is a form of metabolic disorder involving failures of carboxylation enzymes. The deficiency can be in biotinidase or holocarboxylase synthetase . ref name urlMultiple Carboxylase Deficiency cite web url http www.bhj.org journal 2001 4301 jan case 211.htm title Multiple Carboxylase Deficiency format work accessdate ref These conditions respond to biotin . ref name urlDefinition multiple carboxylase deficiency from Online Medical Dictionary cite web url http cancerweb.ncl.ac.uk cgi bin omd?multiple carboxylase deficiency title Definition multiple carboxylase deficiency from Online Medical Dictionary format work accessdate ref Forms include Holocarboxylase synthetase deficiency neonatal Biotinidase deficiency late onset feeding problems,hypotonia,generalised erythematous rash with exfoliation and alopecia,failure to thrive,seizure,coma,developmental delay tomcat urine,metabolic acidosis,ketosis,hyperammonemia References reflist External links http www.pmh.health.wa.gov.au services newborn health professionals disorders mcd prof.htm medicine stub Metabolic disorders of vitamins, coenzymes, and cofactors Category Vitamin, coenzyme, and cofactor metabolism disorders ... more details
enzyme Name geranoyl CoA carboxylase EC number 6.4.1.5 CAS number 37324 35 9 IUBMB EC number 6 4 1 5 GO code 0047925 image width caption In enzymology , a geranoyl CoA carboxylase EC number 6.4.1.5 is an enzyme that catalysis catalyzes the chemical reaction ATP geranoyl CoA HCO sub 3 sub math rightleftharpoons math ADP phosphate 3 4 methylpent 3 en 1 yl pent 2 enedioyl CoA The 3 substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP , geranoyl CoA , and HCO3 , whereas its 3 product chemistry products are adenosine diphosphate ADP , phosphate , and 3 4 methylpent 3 en 1 yl pent 2 enedioyl CoA . This enzyme belongs to the family of ligase s, specifically those forming carbon carbon bonds. The systematic name of this enzyme class is geranoyl CoA carbon dioxide ligase ADP forming . Other names in common use include geranoyl coenzyme A carboxylase , and geranyl CoA carboxylase . It employs one cofactor biochemistry cofactor , biotin . References reflist 1 cite journal author Seubert W, Fass E and Remberger U date 1963 title Untersuchungen uber den bakteriellen Abbau von Isoprenoiden. III Reinigung und Eigenschaften der Geranylcarboxylase journal Biochem. Z. volume 338 pages 265&ndash 275 ligase stub Category EC 6.4.1 Category Biotin enzymes Category Enzymes of unknown structure ... more details
Infobox disease Name 3 Methylcrotonyl CoA carboxylase deficiency ICD10 ICD9 ICDO Image Methylcrotonyl coenzyme A.svg Caption Methylcrotonyl CoA OMIM 210200 OMIM mult OMIM2 210210 MedlinePlus eMedicineSubj eMedicineTopic DiseasesDB 32207 Morefootnotes article date July 2011 3 Methylcrotonyl CoA carboxylase deficiency 3MCC deficiency , also known as 3 Methylcrotonylglycinuria or BMCC deficiency is an genetic disorder inherited disorder in which the body is unable to process certain proteins properly. People with this disorder have inadequate levels of an enzyme that helps break down proteins containing the amino acid leucine . This condition affects an estimated 1 in 50,000 individuals worldwide. Presentation Infants with this disorder appear normal at birth but usually develop signs and symptoms during the first year of life or in early childhood. The characteristic features of this condition, which can range from mild to life threatening, include feeding difficulties, recurrent episodes of vomiting and diarrhea , excessive tiredness lethargy , and weak muscle tone hypotonia . If untreated, this disorder can lead to delayed development, seizures, and coma. Early detection and lifelong management following a low protein diet and using appropriate supplements may prevent many of these complications. In some cases, people with gene mutations that cause 3 methylcrotonyl CoA carboxylase ... Methylcrotonyl CoA Carboxylase Deficiency Evidence for an Allele Specific Dominant Negative Effect .... Individuals with these multiple carboxylase disorders have the same problem with leucine catabolism as those with 3 methylcrotonyl CoA carboxylase deficiency. Genetics The MCCC1 and MCCC2 gene s make protein subunits that come together to form an enzyme called 3 methylcrotonyl CoA carboxylase ... reduces or eliminates the activity of 3 methylcrotonyl CoA carboxylase, the body is unable to process ... Amino acid metabolic pathology DEFAULTSORT 3 Methylcrotonyl Coa Carboxylase ... more details
protein Name methylcrotonoyl Coenzyme A carboxylase 1 alpha caption image width HGNCid 6936 Symbol MCCC1 AltSymbols EntrezGene 56922 OMIM 609010 RefSeq NM 020166 UniProt Q96RQ3 PDB ECnumber 6.4.1.4 Chromosome 3 Arm q Band 27.1 LocusSupplementaryData protein Name methylcrotonoyl Coenzyme A carboxylase 2 beta caption image width HGNCid 6937 Symbol MCCC2 AltSymbols EntrezGene 64087 OMIM 609014 RefSeq NM 022132 UniProt Q9HCC0 PDB ECnumber 6.4.1.4 Chromosome 5 Arm q Band 12 LocusSupplementaryData q13 Methylcrotonyl CoA carboxylase MCC 3 Methylcrotonyl CoA carboxylase , methylcrotonoyl CoA carboxylase is a biotin requiring enzyme located in the mitochondria . MCC uses bicarbonate as a carboxyl group source to catalyze the carboxylation of a carbon adjacent to a carbonyl group performing the 4th step in processing leucine , an essential amino acid. ref Bruice, Paula Y. Organic Chemistry. New Jersey Prentice Hall, 2001. 1010 1011. ref Function During branched chain amino acid degradation, MCC performs a single step in the breakdown of leucine to eventually yield acetyl CoA and acetoacetate. ref Berg, Jeremy M., John L. Tymoczko, Lubert Stryer. Biochemistry. New York Freeman, 2002. 652 653. ref MCC catalyzes the carboxylation of 3 methylcrotonyl CoA to 3 methylglutaconyl CoA . 3 Methylglutaconyl CoA is then hydrated to produce 3 hydroxy 3 methylglutaryl CoA . 3 Hydroxy 3 methylglutaryl CoA is cleaved into two molecules, acetoacetate and acetyl CoA . Point mutations and deletion events in the genes coding for MCC can lead to MCC deficiency , an inborn error of metabolism which usually presents with vomiting, metabolic acidosis , very low plasma glucose concentration, and very low levels of carnitine in plasma. ref Stipanuk, Martha H. Biochemical and Physiological Aspects of Human .... ref References references External links MeshName Methylcrotonoyl CoA carboxylase Carbon carbon ligases Amino acid metabolism enzymes Category EC 6.4.1 de Methylcrotonoyl CoA Carboxylase ja ... more details
enzyme Name Acetyl CoA carboxylase EC number 6.4.1.2 CAS number 9023 93 2 IUBMB EC number 6 4 1 2 GO code 0003989 image width caption Protein Name ACACA Acetyl CoA carboxylase alpha Symbol ACACA AltSymbols ... OMIM 601557 EntrezGene 31 RefSeq NM 198839 UniProt Q13085 Protein Name ACACB Acetyl CoA carboxylase ... CoA carboxylase ACC is a biotin dependent enzyme that catalyzes the irreversible carboxylation of acetyl CoA to produce malonyl CoA through its two catalytic activities, biotin carboxylase BC and carboxyltransferase ... L title Acetyl coenzyme A carboxylase crucial metabolic enzyme and attractive target for drug discovery ..., Boone AN title Isoforms of acetyl CoA carboxylase structures, regulatory properties and metabolic functions ... A, Chirala SS, Wakil SJ title Human acetyl CoA carboxylase characterization, molecular cloning ... acetyl CoA carboxylase gene journal Biochem. J. volume Pt 3 issue pages 915 22 year 1996 series ... by distinct genes. Biotin carboxylase BC activity, biotin carboxyl carrier protein BCCP , and carboxyl ... carboxylase BC , biotin binding BB , carboxyltransferase CT , and ATP binding AB . AB lies within .... ref name pmid15968460 Gallery title Crystallographic structures of E. Coli Acetyl CoA Carboxylase width 270 lines 3 Image Biotin carboxylase rainbow.png Cartoon diagram of Biotin Carboxylase of E.Coli Acetyl CoA Carboxylase Image BCCP.png Biotin Carboxyl Carrier Protein of E. Coli Acetyl CoA Carboxylase Image Carboxytransferase 2F9Y E Coli.png Carboxyltransferase Subunit of E. Coli Acetyl CoA Carboxylase Mechanism The overall reaction of ACAC A,B proceeds by a two step mechanism. ref name ... Biotinoyl domain of human acetyl CoA carboxylase Structural insights into the carboxyl transfer mechanism ... journal author Chou CY, Yu LP, Tong L title Crystal structure of biotin carboxylase in complex with substrates ... CoA carboxylase. The resulting enolate attacks CO sub 2 sub to form malonyl CoA. In a competing mechanism ... HC title Promoter usage determines tissue specific responsiveness of the rat acetyl CoA carboxylase ... more details
PBB geneid 2677 Gamma glutamyl carboxylase is an enzyme that in humans is encoded by the GGCX gene , located on chromosome 2 at 2p12. ref name pmid1749935 cite journal author Wu SM, Cheung WF, Frazier D, Stafford DW title Cloning and expression of the cDNA for human gamma glutamyl carboxylase journal Science volume 254 issue 5038 pages 1634 6 year 1991 month December pmid 1749935 doi 10.1126 science.1749935 url issn ref Function Gamma glutamyl carboxylase is an enzyme that catalyzes the posttranslational modification of vitamin K dependent proteins. Many of these vitamin K dependent proteins are involved in coagulation so the function of the encoded enzyme is essential for hemostasis. ref name entrez cite web title Entrez Gene GGCX url http www.ncbi.nlm.nih.gov sites entrez?Db gene&Cmd ShowDetailView&TermToSearch 2677 accessdate ref Most gla domain containing proteins depend on this carboxylation reaction for posttranslational modification . ref name pmid2145029 cite journal author Brenner ... ref In humans, the gamma glutamyl carboxylase enzyme is most highly expressed in the liver. Catalytic reaction Gamma glutamyl carboxylase oxidizes Vitamin K hydroquinone to Vitamin K 2,3 epoxide, while ... will only proceed if the carboxylase enzyme is able to oxidize vitamin K hydroquinone to vitamin .... ref name pmid3896125 cite journal author Suttie JW title Vitamin K dependent carboxylase journal ... The vitamin K dependent carboxylase journal Thromb. Haemost. volume 87 issue 6 pages 937 46 year 2002 ... mechanism of the vitamin K dependent glutamate carboxylase a computational study journal J Phys ... Berkner KL title The vitamin K dependent carboxylase journal Annu. Rev. Nutr. volume 25 issue 1 ... j.molmed.2004.05.003 url issn cite journal author Berkner KL title The vitamin K dependent carboxylase ... DW title The vitamin K dependent carboxylase journal Thromb. Haemost. volume 87 issue 6 pages ... links MeshName glutamyl carboxylase biochemistry stub NLM content Category EC 6.4 The PBB Controls ... more details
enzyme Name ribulose bisphosphate carboxylase lysine N methyltransferase EC number 2.1.1.127 CAS number 139171 98 5 IUBMB EC number 2 1 1 127 GO code 0030785 image width caption In enzymology , a ribulose bisphosphate carboxylase lysine N methyltransferase EC number 2.1.1.127 is an enzyme that catalysis catalyzes the chemical reaction S adenosyl L methionine ribulose 1,5 bisphosphate carboxylase lysine math rightleftharpoons math S adenosyl L homocysteine ribulose 1,5 bisphosphate carboxylase N sub 6 sub methyl L lysine Thus, the two substrate biochemistry substrates of this enzyme are S Adenosyl methionine S adenosyl methionine and ribulose 1,5 bisphosphate carboxylase lysine , whereas its two product chemistry products are S adenosylhomocysteine and ribulose 1,5 bisphosphate carboxylase N6 methyl L lysine . This enzyme belongs to the family of transferase s, specifically those transferring one carbon group methyltransferases. The systematic name of this enzyme class is S adenosyl L methionine 3 phospho D glycerate carboxy lyase dimerizing lysine N6 methyltransferase . Other names in common use include rubisco methyltransferase , ribulose bisphosphate carboxylase oxygenase N methyltransferase , ribulose 1,5 bisphosphate carboxylase oxygenase large subunit , epsilonN methyltransferase , S adenosyl L methionine 3 phospho D glycerate carboxy lyase , and dimerizing lysine 6 N methyltransferase . Structural studies As of late 2007, 7 tertiary structure structures have been solved for this class of enzymes, with Protein Data Bank PDB accession codes PDB link 1MLV , PDB link 1OZV , PDB link 1P0Y , PDB link 2H21 , PDB link 2H23 , PDB link 2H2E , and PDB link 2H2J . References reflist ... 1,5 bisphosphate carboxylase oxygenase large subunit epsilon N methyltransferase journal Protein ... 1,5 bisphosphate carboxylase oxygenase large subunit epsilon N methyltransferase gene in tobacco ... Ribulose Bisphosphate Carboxylase Lysine N Methyltransferase Category EC 2.1.1 Category Enzymes ... more details
enzyme Name biotin methylcrotonoyl CoA carboxylase ligase EC number 6.3.4.11 CAS number 37318 68 6 IUBMB EC number 6 3 4 11 GO code 0004078 image width caption In enzymology , a biotin methylcrotonoyl CoA carboxylase ligase EC number 6.3.4.11 is an enzyme that catalysis catalyzes the chemical reaction ATP biotin apo 3 methylcrotonoyl CoA carbon dioxide ligase ADP forming math rightleftharpoons math AMP diphosphate 3 methylcrotonoyl CoA carbon dioxide ligase ADP forming The 3 substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP , biotin , and apo 3 methylcrotonoyl CoA carbon dioxide ligase ADP forming , whereas its 3 product chemistry products are adenosine monophosphate AMP , diphosphate , and 3 methylcrotonoyl CoA carbon dioxide ligase ADP forming . This enzyme belongs to the family of ligase s, specifically those forming generic carbon nitrogen bonds. The systematic name of this enzyme class is biotin apo 3 methylcrotonoyl CoA carbon dioxide ligase ADP forming ligase AMP forming . Other names in common use include biotin methylcrotonoyl CoA carboxylase synthetase , biotin beta methylcrotonyl coenzyme A carboxylase synthetase , beta methylcrotonyl coenzyme A holocarboxylase synthetase , and holocarboxylase synthetase . This enzyme participates in biotin metabolism . References reflist 1 cite journal author Hopner T, Knappe J date 1965 title Synthesis of biotin in beta methylcrotonyl CoA carboxylase by holocarboxylase synthetase journal Biochem. Z. volume 342 pages 190&ndash 206 pmid 5867144 issue 2 ligase stub Category EC 6.3.4 Category Enzymes of unknown structure ... more details
int filedesc Information Description en 1 Control of Acetyl CoA Carboxylase. Uploaded PNG because the SVG cannot render correctly. Source Own Author User Jimhsu77479 Jimhsu77479 originally posted by User Alnokta Alnokta Date 2010 11 09 Permission other versions Image Control of Acetyl CoA Carboxylase.svg ImageUpload full int license PD self date November 2010 Copy to Wikimedia Commons bot Fbot priority true ... more details
enzyme Name biotin acetyl CoA carboxylase ligase EC number 6.3.4.15 CAS number 37340 95 7 IUBMB EC number 6 3 4 15 GO code 0004077 image width caption In enzymology , a biotin acetyl CoA carboxylase ligase EC number 6.3.4.15 is an enzyme that catalysis catalyzes the chemical reaction ATP biotin apo acetyl CoA carbon dioxide ligase ADP forming math rightleftharpoons math AMP diphosphate acetyl CoA carbon dioxide ligase ADP forming The 3 substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP , biotin , and apo acetyl CoA carbon dioxide ligase ADP forming , whereas its 3 product chemistry products are adenosine monophosphate AMP , diphosphate , and acetyl CoA carbon dioxide ligase ADP forming . This enzyme belongs to the family of ligase s, specifically those forming generic carbon nitrogen bonds. The systematic name of this enzyme class is biotin apo acetyl CoA carbon dioxide ligase ADP forming ligase AMP forming . Other names in common use include biotin acetyl CoA carboxylase synthetase , biotin acetyl coenzyme A carboxylase synthetase , acetyl coenzyme A holocarboxylase synthetase , acetyl CoA holocarboxylase synthetase , biotin apocarboxylase ligase , Biotin holoenzyme synthetase , and HCS . This enzyme participates in biotin metabolism . Structural studies As of late 2007, 29 tertiary structure structures have been solved for this class of enzymes, with Protein Data Bank PDB accession codes PDB link 1BIA , PDB link 1BIB , PDB link 1HXD , PDB link 1WNL , PDB link 1WPY , PDB link 1WQ7 , PDB link 1WQW , PDB link 1X01 , PDB link 2CGH , PDB link 2DEQ , PDB link 2DJZ , PDB link 2DKG , PDB link 2DTH , PDB link 2DTI , PDB link 2DTO , PDB link 2DVE , PDB link 2DXT , PDB link 2DXU , PDB link 2DZ9 , PDB link 2DZC , PDB link 2E10 , PDB link 2E3Y , PDB link 2E41 , PDB link 2E64 , PDB link 2E65 , PDB link 2EAY , PDB link 2EWN , PDB link 2FYK , and PDB link ... Coenzyme A carboxylase. Role of the prosthetic group in enzyme polymerization journal Biochem. J. volume ... more details
enzyme Name biotin propionyl CoA carboxylase ATP hydrolyzing ligase EC number 6.3.4.10 CAS number 37318 67 5 IUBMB EC number 6 3 4 10 GO code 0004080 image width caption In enzymology , a biotin propionyl CoA carboxylase ATP hydrolysing ligase EC number 6.3.4.10 is an enzyme that catalysis catalyzes the chemical reaction ATP biotin apo propionyl CoA carbon dioxide ligase ADP forming math rightleftharpoons math AMP diphosphate propionyl CoA carbon dioxide ligase ADP forming The 3 substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP , biotin , and apo propionyl CoA carbon dioxide ligase ADP forming , whereas its 3 product chemistry products are adenosine monophosphate AMP , diphosphate , and propionyl CoA carbon dioxide ligase ADP forming . This enzyme belongs to the family of ligase s, specifically those forming generic carbon nitrogen bonds. The systematic name of this enzyme class is biotin apo propanoyl CoA carbon dioxide ligase ADP forming ligase AMP forming . Other names in common use include biotin propionyl CoA carboxylase ATP hydrolysing synthetase , biotin propionyl coenzyme A carboxylase synthetase , and propionyl coenzyme A holocarboxylase synthetase . This enzyme participates in biotin metabolism . References reflist 1 cite journal author Siegel L, Foote JL and Coon MJ date 1965 title The enzymatic synthesis of propionyl coenzyme A holocarboxylase from d biotinyl 5 adenylate and the apocarboxylase journal J. Biol. Chem. volume 240 pages 1025&ndash 1031 pmid 14284697 ligase stub Category EC 6.3.4 Category Enzymes of unknown structure ... more details
PAICS can refer to Philosophy of Artificial Intelligence and Cognitive Science Phosphoribosylaminoimidazolecarboxylase disambig Short pages monitor This long comment was added to the page to prevent it being listed on Special Shortpages. It and the accompanying monitoring template were generated via Template Longcomment. Please do not remove the monitor template without removing the comment as well. ... more details
chembox Verifiedfields changed verifiedrevid 456503689 ImageFile Aminoimidazole ribotide.svg ImageSize IUPACName 5 5 amino imidazole imidazol 1 yl 3,4 dihydroxy oxolane oxolan 2 yl methyl dihydrogen phosphate OtherNames AIR, br 5 5 amino 1 imidazolyl 3,4 dihydroxy 2 tetrahydrofuranyl methyl dihydrogen phosphate Section1 Chembox Identifiers ChemSpiderID Ref chemspidercite correct chemspider ChemSpiderID 141854 StdInChI Ref stdinchicite correct chemspider StdInChI 1S C8H14N3O7P c9 5 1 10 3 11 5 8 7 13 6 12 4 18 8 2 17 19 14,15 16 h1,3 4,6 8,12 13H,2,9H2, H2,14,15,16 t4 ,6 ,7 ,8 m1 s1 StdInChIKey Ref stdinchicite correct chemspider StdInChIKey PDACUKOKVHBVHJ XVFCMESISA N CASNo Ref cascite changed ?? CASNo 25635 88 5 ChEBI Ref ebicite changed EBI ChEBI 28843 KEGG Ref keggcite changed kegg KEGG C03373 PubChem 153 SMILES O P O O OC C H 2O C H n1cncc1N C H O C H 2O MeSHName aminoimidazole ribotide Section2 Chembox Properties Formula C sub 8 sub H sub 14 sub N sub 3 sub O sub 7 sub P MolarMass 295.186 g mol Appearance Density MeltingPt BoilingPt Solubility Section3 Chembox Hazards MainHazards FlashPt Autoignition 5 Phosphoribosyl 5 aminoimidazole or aminoimidazole ribotide is an intermediate in the formation of purine s. Thus, it is an intermediate in the adenine pathway , and is synthesized from 5 phosphoribosylformylglycinamidine by AIR synthetase . See also Phosphoribosylaminoimidazole carboxylase 5 carboxyamino imidazole ribonucleotide synthase 5 carboxyamino imidazole ribonucleotide mutase Nucleotide metabolism intermediates DEFAULTSORT Aminoimidazole ribotide, 5 Category Nucleotides biochemistry stub ... more details
chembox verifiedrevid 399329235 ImageFile 5 phosphoribosyl 4 carboxy 5 aminoimidazole.svg ImageSize IUPACName 5 Amino 1 2 R ,3 R ,4 S ,5 R 3,4 dihydroxy 5 phosphonooxymethyl oxolan 2 yl imidazole 4 carboxylic acid OtherNames Carboxyaminoimidazole ribotide, br Carboxyaminoimidazole ribonucleotide, br CAIR, br 5 amino 1 5 O phosphono small D small ribofuranosyl 1 H imidazole 4 carboxylic acid Section1 Chembox Identifiers ChemSpiderID Ref chemspidercite correct chemspider ChemSpiderID 144983 InChI 1 C9H14N3O9P c10 7 4 9 15 16 11 2 12 7 8 6 14 5 13 3 21 8 1 20 22 17,18 19 h2 3,5 6,8,13 14H,1,10H2, H,15,16 H2,17,18,19 t3 ,5 ,6 ,8 m1 s1 InChIKey XFVULMDJZXYMSG ZIYNGMLEBD SMILES1 O P O O OC C H 2O C H n1cnc C O O c1N C H O C H 2O StdInChI Ref stdinchicite correct chemspider StdInChI 1S C9H14N3O9P c10 7 4 9 15 16 11 2 12 7 8 6 14 5 13 3 21 8 1 20 22 17,18 19 h2 3,5 6,8,13 14H,1,10H2, H,15,16 H2,17,18,19 t3 ,5 ,6 ,8 m1 s1 StdInChIKey Ref stdinchicite correct chemspider StdInChIKey XFVULMDJZXYMSG ZIYNGMLESA N CASNo 6001 14 5 PubChem 165388 ChEBI 28413 SMILES C1 NC C N1 C H 2 C H C H C H O2 COP O O O O O N C O O Section2 Chembox Properties Formula C sub 9 sub H sub 14 sub N sub 3 sub O sub 9 sub P MolarMass 339.196 g mol Appearance Density MeltingPt BoilingPt Solubility Section3 Chembox Hazards MainHazards FlashPt Autoignition 5 Phosphoribosyl 4 carboxy 5 aminoimidazole or CAIR is an Reaction intermediate intermediate in the formation of purine s. It is formed by phosphoribosylaminoimidazole carboxylase . Nucleotide metabolism intermediates DEFAULTSORT Phosphoribosyl 4 carboxy 5 aminoimidazole, 5 organic compound stub Category Nucleotides ... more details
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