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Protein-coding gene in the species Homo sapiens
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
GCSH
Medical condition
encephalopathy, which include GLDC (chromosome 9), AMT (chromosome 3), and GCSH (chromosome 16). There is a fourth subunit in the GCS, Dihydrolipoamide dehydrogenase
Glycine_encephalopathy
Chemical compound
dehydrogenase complex (BCKDC) Leucine, isoleucine and valine degradation H-protein GCSH glycine cleavage system (GCS) Glycine and serine metabolism, folate metabolism
Lipoic_acid
(types A and B) 1:150,000 (type C) Nonketotic hyperglycinemia GLDC, AMT, GCSH recessive 1:60,000 Nonsyndromic deafness Noonan syndrome PTPN11, KRAS, SOS1
List_of_genetic_disorders
Myosin Binding Protein H (MYBPH)
Not listed 0.984 - Textmining Catalyzes the degradation of glycine. STRING GCSH Glycine Cleavage System H Protein Not listed 0.957 - Textmining Catalyzes
MYBPH
Enzymes that break down glycine
phosphate) T-protein (GCST or AMT) EC 2.1.2.10 aminomethyltransferase H-protein (GCSH) is modified with lipoic acid and interacts with all other components in
Glycine_cleavage_system
German biochemist and molecular biologist
member of the German Council of Science and Humanities (GCSH) and was elected as chair of the GCSH research commission in 2008. In 2005, Beisiegel was appointed
Ulrike_Beisiegel
Protein-coding gene in the species Homo sapiens
accounting for 70%-75% of disease), AMT (accounting for ~20% of disease), and GCSH (encoding the H-protein component of the GCS complex and accounting for <1%
Aminomethyltransferase
307030; GK Glycine encephalopathy; 605899; AMT Glycine encephalopathy; 605899; GCSH Glycine encephalopathy; 605899; GLDC Glycine N-methyltransferase deficiency;
List_of_OMIM_disorder_codes
Protein-coding gene in the species Homo sapiens
PMID 11286506. Kure S, Kojima K, Ichinohe A, et al. (2002). "Heterozygous GLDC and GCSH gene mutations in transient neonatal hyperglycinemia". Ann. Neurol. 52 (5):
Glycine dehydrogenase (decarboxylating)
Glycine_dehydrogenase_(decarboxylating)
Q6ZNI0 6143 GCSAM HGNC:20253; Q8N6F7 6144 GCSAML HGNC:29583; Q5JQS6 6145 GCSH HGNC:4208; P23434 6146 GDA HGNC:4212; Q9Y2T3 6147 GDAP1 HGNC:15968; Q8TB36
List of human protein-coding genes 3
List_of_human_protein-coding_genes_3
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