Cause of nonketotic hyperglycinemia

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{"type":"standard","title":"GCSH","displaytitle":"GCSH","namespace":{"id":0,"text":""},"wikibase_item":"Q18025700","titles":{"canonical":"GCSH","normalized":"GCSH","display":"GCSH"},"pageid":15214505,"thumbnail":{"source":"https://upload.wikimedia.org/wikipedia/commons/thumb/2/2d/Ideogram_human_chromosome_16.svg/330px-Ideogram_human_chromosome_16.svg.png","width":320,"height":128},"originalimage":{"source":"https://upload.wikimedia.org/wikipedia/commons/thumb/2/2d/Ideogram_human_chromosome_16.svg/474px-Ideogram_human_chromosome_16.svg.png","width":474,"height":189},"lang":"en","dir":"ltr","revision":"1194310894","tid":"6ce5de0c-ae09-11ee-88c0-5372c2228778","timestamp":"2024-01-08T09:36:40Z","description":"Protein-coding gene in the species Homo sapiens","description_source":"local","content_urls":{"desktop":{"page":"https://en.wikipedia.org/wiki/GCSH","revisions":"https://en.wikipedia.org/wiki/GCSH?action=history","edit":"https://en.wikipedia.org/wiki/GCSH?action=edit","talk":"https://en.wikipedia.org/wiki/Talk:GCSH"},"mobile":{"page":"https://en.m.wikipedia.org/wiki/GCSH","revisions":"https://en.m.wikipedia.org/wiki/Special:History/GCSH","edit":"https://en.m.wikipedia.org/wiki/GCSH?action=edit","talk":"https://en.m.wikipedia.org/wiki/Talk:GCSH"}},"extract":"Glycine cleavage system H protein, mitochondrial is a protein that in humans is encoded by the GCSH gene. Degradation of glycine is brought about by the glycine cleavage system (GCS), which is composed of 4 protein components: P protein, H protein, T protein, and L protein. The H protein shuttles the methylamine group of glycine from the P protein to the T protein. The protein encoded by GCSH gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding, have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.","extract_html":"

Glycine cleavage system H protein, mitochondrial is a protein that in humans is encoded by the GCSH gene. Degradation of glycine is brought about by the glycine cleavage system (GCS), which is composed of 4 protein components: P protein, H protein, T protein, and L protein. The H protein shuttles the methylamine group of glycine from the P protein to the T protein. The protein encoded by GCSH gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding, have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.

"}

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