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FLASH GENE
Symbol GCSH contributors: mct - updated : 07-12-2016
HGNC name glycine cleavage system protein H (aminomethyl carrier)
HGNC id 4208
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a N terrminal mitochondrial target sequence (48aa)
  • a lipoic acid binding site
    • mono polymer complex
    isoforms Precursor mature protein of 125AA
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,mitochondria,matrix
    basic FUNCTION
  • amino methyl carrier
  • transfer of methylamine group of glycine from P to T protein, catalyzing the second step of the glycine cleavage system
  • its function is involved in the pathogenesis and diagnosis of glycine encephalopathy
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism aminoacid
    signaling
    a component
  • glycine cleavage system complex
    • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • plausible function of GCSH is to interact with the L-protein, which is also part of the l-ketoglutarate dehydrogenase complex present in fibroblasts
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) GCE1
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS