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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 [protéine H du système de clivage de la glycine]-S-aminométhyl-N6-dihydrolipoyl-L-lysine + CO2.

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

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    • (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, no 3,‎ , p. 801–6 (PMID 2268343, DOI 10.1016/S0006-291X(05)80858-7)
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    • (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, no 2,‎ , 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, no 3,‎ , p. 298–305 (PMID 10798358, DOI 10.1007/s004390051041)
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    • (en) Kure S, Kojima K, Ichinohe A, et al., « Heterozygous GLDC and GCSH gene mutations in transient neonatal hyperglycinemia », Ann. Neurol., vol. 52, no 5,‎ , 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, no 26,‎ , 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, no 5,‎ , 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, no 7,‎ , 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, no 8,‎ , 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, no 5,‎ , p. 230–4 (PMID 15864413, DOI 10.1007/s10038-005-0243-y)
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