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. Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH).
Western Blot: HEK-293 Cells, 1:500-1:5000; IHC: Human Kidney Tissue, 1:20-1:200; IF: MCF-7 Cells, 1:10-1:100
Type: Primary
Antigen: GCSH
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat