Recombinant Human GCSH Protein

重组人GCSH蛋白 | 重组人甘氨酸断裂系统蛋白H

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 |

rp188394-10μg

10μg

无载体

≥95%(SDS-PAGE)

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).

重组表达

14.8 kDa

N-His

14.0 kDa, under reducing conditions; 14.0 kDa, under non-reducing conditions.

E. coli Accession #: P23434 | E. coli

<1.0 EU/μg

人(Human)

49-173 aa

MHHHHHHSVRKFTEKHEWVTTENGIGTVGISNFAQEALGDVVYCSLPEVGTKLNKQDEFGALESVKAASELYSPLSGEVTEINEALAENPGLVNKSCYEDGWLIKMTLSNPSELDELMSEEAYEKYIKSIEE

No

Yes