About: GCSH

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Glycine cleavage system H protein, mitochondrial (abbreviated as GCSH) 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 (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein; this protein), T protein (a tetrahydrofolate-requiring aminomethyltransferase enzyme), and L protein (a lipoamide dehydrogenase). 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 trans

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  • Glycine cleavage system H protein, mitochondrial (abbreviated as GCSH) 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 (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein; this protein), T protein (a tetrahydrofolate-requiring aminomethyltransferase enzyme), and L protein (a lipoamide dehydrogenase). 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. (en)
  • La protéine H du système de clivage de la glycine est l'une des quatre composantes du système de clivage de la glycine, un complexe enzymatique qui intervient pour dégrader les excès de glycine. Il s'agit d'une protéine portant un résidu d'acide lipoïque dont la fonction est de transporter le groupe méthylamine de la glycine depuis la glycine décarboxylase jusqu'à la dihydrolipoyl déshydrogénase. (fr)
  • GCSH (англ. Glycine cleavage system protein H) – білок, який кодується однойменним геном, розташованим у людей на короткому плечі 16-ї хромосоми. Довжина поліпептидного ланцюга білка становить 173 амінокислот, а молекулярна маса — 18 885. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин Локалізований у мітохондрії. (uk)
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  • La protéine H du système de clivage de la glycine est l'une des quatre composantes du système de clivage de la glycine, un complexe enzymatique qui intervient pour dégrader les excès de glycine. Il s'agit d'une protéine portant un résidu d'acide lipoïque dont la fonction est de transporter le groupe méthylamine de la glycine depuis la glycine décarboxylase jusqu'à la dihydrolipoyl déshydrogénase. (fr)
  • GCSH (англ. Glycine cleavage system protein H) – білок, який кодується однойменним геном, розташованим у людей на короткому плечі 16-ї хромосоми. Довжина поліпептидного ланцюга білка становить 173 амінокислот, а молекулярна маса — 18 885. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин Локалізований у мітохондрії. (uk)
  • Glycine cleavage system H protein, mitochondrial (abbreviated as GCSH) 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 (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein; this protein), T protein (a tetrahydrofolate-requiring aminomethyltransferase enzyme), and L protein (a lipoamide dehydrogenase). 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 trans (en)
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  • GCSH (en)
  • Protéine H du système de clivage de la glycine (fr)
  • GCSH (uk)
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