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Mouse Anti-GCSH Recombinant Antibody (CBLG1-819) (CBMAB-G2678-LY)

This product is antibody recognizes GCSH. The antibody CBLG1-819 immunoassay techniques such as: S-ELISA, ELISA, WB.
See all GCSH antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBLG1-819
Antibody Isotype
IgG1, κ
Application
S-ELISA, ELISA, WB

Basic Information

Immunogen
GCSH (AAH00790.1, 1 a.a. ~ 173 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
glycine cleavage system protein H (aminomethyl carrier)
Introduction
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]
Entrez Gene ID
2653
UniProt ID
P23434
Alternative Names
Glycine Cleavage System Protein H; Lipoic Acid-Containing Protein; Glycine Cleavage System Protein H (Aminomethyl Carrier); Glycine Cleavage System H Protein; Mitochondrial; Mitochondrial Glycine Cleavage System H-Protein; GCE; NKH;
Function
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).
Biological Process
Glycine catabolic process Source: ProtInc
Glycine decarboxylation via glycine cleavage system Source: GO_Central
Protein lipoylation Source: UniProtKB
Cellular Location
Mitochondrion
Involvement in disease
Non-ketotic hyperglycinemia (NKH):
Autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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