Glycine cleavage system H protein, mitochondrial

NameGlycine cleavage system H protein, mitochondrial
SynonymsLipoic acid-containing protein
Gene NameGCSH
OrganismHuman
Amino acid sequence
>lcl|BSEQ0011658|Glycine cleavage system H protein, mitochondrial
MALRVVRSVRALLCTLRAVPSPAAPCPPRPWQLGVGAVRTLRTGPALLSVRKFTEKHEWV
TTENGIGTVGISNFAQEALGDVVYCSLPEVGTKLNKQDEFGALESVKAASELYSPLSGEV
TEINEALAENPGLVNKSCYEDGWLIKMTLSNPSELDELMSEEAYEKYIKSIEE
Number of residues173
Molecular Weight18884.37
Theoretical pI4.56
GO Classification
Functions
    aminomethyltransferase activity
Processes
    glycine decarboxylation via glycine cleavage system
cellular nitrogen compound metabolic process
small molecule metabolic process
glycine catabolic process
glyoxylate metabolic process
methylation
Components
    mitochondrion
mitochondrial matrix
glycine cleavage complex
General FunctionAminomethyltransferase activity
Specific FunctionThe glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST).
Transmembrane Regions
GenBank Protein ID184348
UniProtKB IDP23434
UniProtKB Entry NameGCSH_HUMAN
Cellular LocationMitochondrion
Gene sequence
>lcl|BSEQ0011659|Glycine cleavage system H protein, mitochondrial (GCSH)
ATGGCGCTGCGAGTGGTGCGGAGCGTGCGGGCCCTGCTCTGCACCCTGCGCGCGGTCCCG
TCACCCGCCGCGCCCTGCCCGCCGAGGCCCTGGCAGCTGGGGGTGGGCGCCGTCCGTACG
CTGCGCACTGGACCCGCTCTGCTCTCGGTGCGTAAATTCACAGAGAAACACGAATGGGTA
ACAACAGAAAATGGCATTGGAACAGTGGGAATCAGCAATTTTGCACAGGAAGCGTTGGGA
GATGTTGTTTATTGTAGTCTCCCTGAAGTTGGGACAAAATTGAACAAACAAGATGAGTTT
GGTGCTTTGGAAAGTGTGAAAGCTGCTAGTGAACTCTATTCTCCTTTATCAGGAGAAGTA
ACTGAAATTAATGAAGCTCTTGCAGAAAATCCAGGACTTGTAAACAAATCTTGTTATGAA
GATGGTTGGCTGATCAAGATGACACTGAGTAACCCTTCAGAACTAGATGAACTTATGAGT
GAAGAAGCATATGAGAAATACATAAAATCTATTGAGGAGTGA
GenBank Gene IDM69175
GeneCard IDNone
GenAtlas IDGCSH
HGNC IDHGNC:4208
Chromosome Location16
Locus16q23.2
References
  1. Fujiwara K, Okamura-Ikeda K, Hayasaka K, Motokawa Y: The primary structure of human H-protein of the glycine cleavage system deduced by cDNA cloning. Biochem Biophys Res Commun. 1991 Apr 30;176(2):711-6.[2025283 ]
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  3. Koyata H, Hiraga K: The glycine cleavage system: structure of a cDNA encoding human H-protein, and partial characterization of its gene in patients with hyperglycinemias. Am J Hum Genet. 1991 Feb;48(2):351-61.[1671321 ]
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Related FRC

FRCD ID Name Exact Mass Structure




F05337



Glycine


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