Glycine cleavage system H protein, mitochondrial

NameGlycine cleavage system H protein, mitochondrial
Synonyms
  • Lipoic 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
  • cellular nitrogen compound metabolic process
  • small molecule metabolic process
  • glycine catabolic process
  • glyoxylate metabolic process
  • methylation
  • glycine decarboxylation via glycine cleavage system
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).
Pfam Domain Function
Transmembrane RegionsNot Available
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 IDNot Available
GenAtlas IDGCSH
HGNC IDHGNC:4208
Chromosome Location16
Locus16q23.2
References
  1. 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
  2. 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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