GCSH
Reactivity: Human
WB, IF (cc), IF (p)
Host: Rabbit
Polyclonal
AbBy Fluor® 680
Application Notes
IHC 1:50-1:200, ELISA 1:5000-1:10000
Restrictions
For Research Use only
Format
Liquid
Concentration
1.02 mg/mL
Buffer
PBS with 0.05 % Sodium azide and 40 % Glycerol, pH 7.4
Preservative
Sodium azide
Precaution of Use
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage
-20 °C
Storage Comment
Store at -20°C. Avoid freeze / thaw cycles.
Target
GCSH
(Glycine Cleavage System H Protein (GCSH))
Alternative Name
GCSH
Background
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.