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|Author:||Mark A Johnson|
Amino Acid starvation has opposite effects on mitochondrial and cytosolic protein synthesis.
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GCSH(Glycine cleavage system H protein, mitochondrial) is a component of the glycine cleavage system loosely associated with the mitochondrial inner membrane and has lipoic acid as a prosthetic group. The full-length GCSH cDNA encoding a precursor protein of 173 amino acids and a mature protein of 125 amino acids.The lipoylation of H-protein occurs in mitochondria which probably contain an activated form of lipoic acid as well as other components required for the transfer of lipoic acid to the protein(PMID:2211640). Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH).