Glycine décarboxylase

La glycine décarboxylase, ou glycine déshydrogénase (décarboxylante), est une oxydoréductase qui catalyse la réaction : glycine + [protéine H du système de clivage de la glycine]-N⁶-lipoyl-L-lysine $\rightleftharpoons$ [protéine H du système de clivage de la glycine]-S-aminométhyl-N⁶-dihydrolipoyl-L-lysine + CO₂.

Glycine déshydrogénase (décarboxylante)

Données clés
N° EC	EC 1.4.4.2
N° CAS	37259-67-9
Cofacteur(s)	PLP

Activité enzymatique
IUBMB	Entrée IUBMB
IntEnz	Vue IntEnz
BRENDA	Entrée BRENDA
KEGG	Entrée KEGG
MetaCyc	Voie métabolique
PRIAM	Profil
PDB	RCSB PDB PDBe PDBj PDBsum
GO	AmiGO / EGO

Cette enzyme est l'un des quatre composants du système de clivage de la glycine, qui intervient en réponse à des taux élevés de glycine, aussi bien chez les humains que chez les plantes où elle intervient dans la photorespiration au sein des chloroplastes.

Notes et références

(en) Hiraga K, Kikuchi G, « The mitochondrial glycine cleavage system. Functional association of glycine decarboxylase and aminomethyl carrier protein », J. Biol. Chem., vol. 255, n^o 24,‎ 1980, p. 11671–6 (PMID 7440563)
(en) Perham RN, « Swinging arms and swinging domains in multifunctional enzymes: catalytic machines for multistep reactions », Annu. Rev. Biochem., vol. 69,‎ 2000, p. 961–1004 (PMID 10966480, DOI 10.1146/annurev.biochem.69.1.961)
(en) Broadwater JA, Haas JA, Fox BG, Booker SJ, « Expression, purification, and physical characterization of Escherichia coli lipoyl(octanoyl)transferase », Protein. Expr. Purif., vol. 39, n^o 2,‎ 2005, p. 269–82 (PMID 15642479, DOI 10.1016/j.pep.2004.10.021)*Applegarth DA, Toone JR, « Nonketotic hyperglycinemia (glycine encephalopathy): laboratory diagnosis », Mol. Genet. Metab., vol. 74, n^os 1–2,‎ 2001, p. 139–46 (PMID 11592811, DOI 10.1006/mgme.2001.3224)
(en) Kure S, Takayanagi M, Narisawa K, et al., « Identification of a common mutation in Finnish patients with nonketotic hyperglycinemia », J. Clin. Invest., vol. 90, n^o 1,‎ 1992, p. 160–4 (PMID 1634607, PMCID 443076, DOI 10.1172/JCI115831)
(en) Sakakibara T, Koyata H, Ishiguro Y, et al., « One of the two genomic copies of the glycine decarboxylase cDNA has been deleted at a 5' region in a patient with nonketotic hyperglycinemia », Biochem. Biophys. Res. Commun., vol. 173, n^o 3,‎ 1991, p. 801–6 (PMID 2268343, DOI 10.1016/S0006-291X(05)80858-7)
(en) Burton BK, Pettenati MJ, Block SM, et al., « Nonketotic hyperglycinemia in a patient with the 9p- syndrome », Am. J. Med. Genet., vol. 32, n^o 4,‎ 1989, p. 504–5 (PMID 2773994, DOI 10.1002/ajmg.1320320416)
(en) Hayasaka K, Kochi H, Hiraga K, Kikuchi G, « Purification and properties of glycine decarboxylase, a component of the glycine cleavage system, from rat liver mitochondria and immunochemical comparison of this enzyme from various sources », J. Biochem., vol. 88, n^o 4,‎ 1981, p. 1193–9 (PMID 6778858)
(en) Hiraga K, Kochi H, Hayasaka K, et al., « Defective glycine cleavage system in nonketotic hyperglycinemia. Occurrence of a less active glycine decarboxylase and an abnormal aminomethyl carrier protein », J. Clin. Invest., vol. 68, n^o 2,‎ 1981, p. 525–34 (PMID 6790577, PMCID 370827, DOI 10.1172/JCI110284)
(en) Takayanagi M, Kure S, Sakata Y, et al., « Human glycine decarboxylase gene (GLDC) and its highly conserved processed pseudogene (psiGLDC): their structure and expression, and the identification of a large deletion in a family with nonketotic hyperglycinemia », Hum. Genet., vol. 106, n^o 3,‎ 2000, p. 298–305 (PMID 10798358, DOI 10.1007/s004390051041)
(en) Toone JR, Applegarth DA, Coulter-Mackie MB, James ER, « Biochemical and molecular investigations of patients with nonketotic hyperglycinemia », Mol. Genet. Metab., vol. 70, n^o 2,‎ 2000, p. 116–21 (PMID 10873393, DOI 10.1006/mgme.2000.3000)
(en) Toone JR, Applegarth DA, Coulter-Mackie MB, James ER, « Recurrent mutations in P- and T-proteins of the glycine cleavage complex and a novel T-protein mutation (N145I): a strategy for the molecular investigation of patients with nonketotic hyperglycinemia (NKH) », Mol. Genet. Metab., vol. 72, n^o 4,‎ 2001, p. 322–5 (PMID 11286506, DOI 10.1006/mgme.2001.3158)
(en) Kure S, Kojima K, Ichinohe A, et al., « Heterozygous GLDC and GCSH gene mutations in transient neonatal hyperglycinemia », Ann. Neurol., vol. 52, n^o 5,‎ 2002, p. 643–6 (PMID 12402263, DOI 10.1002/ana.10367)
(en) Strausberg RL, Feingold EA, Grouse LH, et al., « Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences », Proc. Natl. Acad. Sci. U.S.A., vol. 99, n^o 26,‎ 2003, p. 16899–903 (PMID 12477932, PMCID 139241, DOI 10.1073/pnas.242603899)
(en) Toone JR, Applegarth DA, Laliberte G, « Gene Symbol: GLDC. Disease: NKH glycine encephalopathy », Hum. Genet., vol. 113, n^o 5,‎ 2003, p. 465 (PMID 14552331, DOI 10.1007/s00439-003-1014-5)
(en) Dinopoulos A, Kure S, Chuck G, et al., « Glycine decarboxylase mutations: a distinctive phenotype of nonketotic hyperglycinemia in adults », Neurology, vol. 64, n^o 7,‎ 2006, p. 1255–7 (PMID 15824356, DOI 10.1212/01.WNL.0000156800.23776.40)
(en) Flusser H, Korman SH, Sato K, et al., « Mild glycine encephalopathy (NKH) in a large kindred due to a silent exonic GLDC splice mutation », Neurology, vol. 64, n^o 8,‎ 2006, p. 1426–30 (PMID 15851735, DOI 10.1212/01.WNL.0000158475.12907.D6)
(en) Boneh A, Korman SH, Sato K, et al., « A single nucleotide substitution that abolishes the initiator methionine codon of the GLDC gene is prevalent among patients with glycine encephalopathy in Jerusalem », J. Hum. Genet., vol. 50, n^o 5,‎ 2005, p. 230–4 (PMID 15864413, DOI 10.1007/s10038-005-0243-y)
(en) Kimura K, Wakamatsu A, Suzuki Y, et al., « Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes », Genome Res., vol. 16, n^o 1,‎ 2006, p. 55–65 (PMID 16344560, PMCID 1356129, DOI 10.1101/gr.4039406)
(en) Korman SH, Wexler ID, Gutman A, et al., « Treatment from birth of nonketotic hyperglycinemia due to a novel GLDC mutation », Ann. Neurol., vol. 59, n^o 2,‎ 2006, p. 411–5 (PMID 16404748, DOI 10.1002/ana.20759)

Cet article est issu de wikipedia. Text licence: CC BY-SA 4.0, Des conditions supplémentaires peuvent s’appliquer aux fichiers multimédias.