glycine cleavage system protein H (aminomethyl carrier), Polyclonal Antibody

glycine cleavage system protein H (aminomethyl carrier); GCSH; GCE; NKH

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Antigen Affinity Purified

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-GCSH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

18,885 Da[Similar Products]

glycine cleavage system protein H (aminomethyl carrier)

glycine cleavage system H protein, mitochondrial; lipoic acid-containing protein; mitochondrial glycine cleavage system H-protein

Glycine cleavage system H protein, mitochondrial

GCSH_HUMAN

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]

GCSH: The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein. Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH); also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Belongs to the GcvH family.

Chromosomal Location of Human Ortholog: 16q23.2

Cellular Component: mitochondrion; glycine cleavage complex

Molecular Function: enzyme binding; aminomethyltransferase activity

Biological Process: methylation; glycine decarboxylation via glycine cleavage system; glycine catabolic process

Disease: Glycine Encephalopathy

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