GCE | GCSH | GCSH_HUMAN | Glycine cleavage system H protein | Glycine cleavage system H protein mitochondrial | Glycine cleavage system protein H | Glycine cleavage system protein H (aminomethyl carrier) | Lipoic acid containing protein | mitochondrial |
规格或纯度
无载体,His标签,≥95%(SDS-PAGE),见COA
生化机理
There are four mitochondrial proteins composing the enzyme system for cleavage of glycine (glycine cleavage system): P protein, H protein, T protein, and L protein. GCSH is the H protein. GCSH transfers the methylamine group of glycine from the P protein to the T protein. Mutations in this gene results in nonketotic hyperglycinemia (NKH). The 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). Has a pivotal role in the lipoylation of enzymes involved in cellular energetics such as the mitochondrial dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex (DLAT), and the mitochondrial dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex (DLST) (PubMed:36190515).
Store at -20°C for 1 year. Avoid freeze/thaw cycle.
图片
Recombinant Human GCSH Protein (rp188394) - SDS-PAGE 3 μg/lane of Recombinant Human GCSH Protein was resolved with SDS-PAGE under reducing (R) and non-reducing (N) conditions and visualized by Coomassie® Blue staining, showing a band at 14.0 kDa.
ZJ24F1214745
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