Specifications
Immunogen
GCSH (AAH00790.1, 1 a.a. ~ 173 a.a) full-length recombinant protein with GST tag. The sequence is MALRVVRSVRALLCTLRAVPLPAAPCPPRPWQLGVGAVRTLRTGPALLSVRKFTEKHEWV TTENGIGTVGISNFAQEALGDVVYCSLPEVGTKLNKQDEFGALESVKAASELYSPLSGEV TEINEALAENPGLVNKSCYEDGWLIKMTLSNPSELDELMSEEAYEKYIKSIEE
Target
Alternative Names
GCSH; glycine cleavage system protein H (aminomethyl carrier); GCE; NKH; glycine cleavage system H protein, mitochondrial; lipoic acid-containing protein; mitochondrial glycine cleavage system H-protein;
Product Background
Antigen Description
Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.[provided by RefSeq, Jan 2010]
Pathway
Glycine, serine and threonine metabolism; Glyoxylate and dicarboxylate metabolism.
Citations
Publication ()
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