NP_598282.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Related Accession #
Molecular Weight
18,485 Da
NCBI Official Full Name
glycine cleavage system H protein, mitochondrial
NCBI Official Synonym Full Names
glycine cleavage system protein H (aminomethyl carrier)
NCBI Protein Information
glycine cleavage system H protein, mitochondrial
UniProt Protein Name
Glycine cleavage system H protein, mitochondrial
UniProt Synonym Protein Names
Lipoic acid-containing protein
UniProt Entry Name
GCSH_RAT
NCBI Summary for GCSH
component of the mitochondrial multienzyme glycine cleavage system (GCS); deficiency of the GCS in human causes nonketotic hyperglycinemia (NKH), an inborn error of glycine metabolism [RGD, Feb 2006]
UniProt Comments for GCSH
GCSH: The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein. Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH); also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Belongs to the GcvH family.
Cellular Component: mitochondrion; glycine cleavage complex
Molecular Function: enzyme binding; aminomethyltransferase activity
Biological Process: methylation; glycine decarboxylation via glycine cleavage system
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Products associated with GCSH sirna
Pathways associated with GCSH sirna
Diseases associated with GCSH sirna
Organs/Tissues associated with GCSH sirna
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