GCSH

GCSH

Glycine cleavage system protein H (aminomethyl carrier), also known as GCSH, is a human gene.cite web | title = Entrez Gene: GCSH glycine cleavage system protein H (aminomethyl carrier)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2653| accessdate = ]

PBB_Summary
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summary_text = The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase; MIM 238300), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme; MIM 238310), and L protein (a lipoamide dehydrogenase; MIM 238331). Glycine encephalopathy (GCE; MIM 605899), also called nonketotic hyperglycinemia (NKH), may be due to a defect in any one of these enzymes. [supplied by OMIM] cite web | title = Entrez Gene: GCSH glycine cleavage system protein H (aminomethyl carrier)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2653| accessdate = ]

References

Further reading

PBB_Further_reading
citations =
*cite journal | author=Koyata H, Hiraga K |title=The glycine cleavage system: structure of a cDNA encoding human H-protein, and partial characterization of its gene in patients with hyperglycinemias. |journal=Am. J. Hum. Genet. |volume=48 |issue= 2 |pages= 351–61 |year= 1991 |pmid= 1671321 |doi=
*cite journal | author=Fujiwara K, Okamura-Ikeda K, Hayasaka K, Motokawa Y |title=The primary structure of human H-protein of the glycine cleavage system deduced by cDNA cloning. |journal=Biochem. Biophys. Res. Commun. |volume=176 |issue= 2 |pages= 711–6 |year= 1991 |pmid= 2025283 |doi=
*cite journal | author=Hiraga K, Kure S, Yamamoto M, "et al." |title=Cloning of cDNA encoding human H-protein, a constituent of the glycine cleavage system. |journal=Biochem. Biophys. Res. Commun. |volume=151 |issue= 2 |pages= 758–62 |year= 1988 |pmid= 3348809 |doi=
*cite journal | author=Gründig E, Birnbaumer E, Hawrylewicz A |title=Influence of phenothiazines or reserpine on the formation of 14C-glycine from U-14C-serine. |journal=Enzyme |volume=26 |issue= 1 |pages= 43–8 |year= 1981 |pmid= 6111451 |doi=
*cite journal | author=Hiraga K, Kochi H, Hayasaka K, "et al." |title=Defective glycine cleavage system in nonketotic hyperglycinemia. Occurrence of a less active glycine decarboxylase and an abnormal aminomethyl carrier protein. |journal=J. Clin. Invest. |volume=68 |issue= 2 |pages= 525–34 |year= 1981 |pmid= 6790577 |doi=
*cite journal | author=Kure S, Kojima K, Kudo T, "et al." |title=Chromosomal localization, structure, single-nucleotide polymorphisms, and expression of the human H-protein gene of the glycine cleavage system (GCSH), a candidate gene for nonketotic hyperglycinemia. |journal=J. Hum. Genet. |volume=46 |issue= 7 |pages= 378–84 |year= 2001 |pmid= 11450847 |doi=
*cite journal | author=Kure S, Kojima K, Ichinohe A, "et al." |title=Heterozygous GLDC and GCSH gene mutations in transient neonatal hyperglycinemia. |journal=Ann. Neurol. |volume=52 |issue= 5 |pages= 643–6 |year= 2002 |pmid= 12402263 |doi= 10.1002/ana.10367
*cite journal | author=Strausberg RL, Feingold EA, Grouse LH, "et al." |title=Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=99 |issue= 26 |pages= 16899–903 |year= 2003 |pmid= 12477932 |doi= 10.1073/pnas.242603899
*cite journal | author=Gerhard DS, Wagner L, Feingold EA, "et al." |title=The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). |journal=Genome Res. |volume=14 |issue= 10B |pages= 2121–7 |year= 2004 |pmid= 15489334 |doi= 10.1101/gr.2596504
*cite journal | author=Rual JF, Venkatesan K, Hao T, "et al." |title=Towards a proteome-scale map of the human protein-protein interaction network. |journal=Nature |volume=437 |issue= 7062 |pages= 1173–8 |year= 2005 |pmid= 16189514 |doi= 10.1038/nature04209

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