Full Product Name
GCSH Antibody (N-term)
Product Synonym Names
Glycine cleavage system H protein; mitochondrial; Lipoic acid-containing protein; GCSH
Product Gene Name
anti-GCSH antibody
[Similar Products]
Antibody/Peptide Pairs
GCSH peptide (MBS9222133) is used for blocking the activity of GCSH antibody (MBS9203149)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Positions
27-56
3D Structure
ModBase 3D Structure for P23434
Species Reactivity
Human (Predicted Reactivity: Bovine, Rabbit)
Specificity
This GCSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 27-56 amino acids from the N-terminal region of human GCSH.
Purity/Purification
Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
Form/Format
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
Concentration
Vial Concentration: 0.5 (lot specific)
Antigen Type
Synthetic Peptide
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.
Other Notes
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.
Related Product Information for
anti-GCSH antibody
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.
Product Categories/Family for anti-GCSH antibody
Metabolism; Signal Transduction
Applications Tested/Suitable for anti-GCSH antibody
Western Blot (WB), ELISA (EIA)
Application Notes for anti-GCSH antibody
WB~~1:1000
Western Blot (WB) of anti-GCSH antibody
GCSH Antibody (N-term) western blot analysis in T47D cell line lysates (35ug/lane).This demonstrates the GCSH antibody detected the GCSH protein (arrow).
NCBI/Uniprot data below describe general gene information for GCSH. It may not necessarily be applicable to this product.
NCBI Accession #
NP_004474.2
[Other Products]
NCBI GenBank Nucleotide #
NM_004483.4
[Other Products]
UniProt Primary Accession #
P23434
[Other Products]
UniProt Secondary Accession #
Q9H1E9[Other Products]
UniProt Related Accession #
P23434[Other Products]
NCBI Official Full Name
glycine cleavage system H protein, mitochondrial
NCBI Official Synonym Full Names
glycine cleavage system protein H (aminomethyl carrier)
NCBI Official Symbol
GCSH [Similar Products]
NCBI Official Synonym Symbols
GCE; NKH
[Similar Products]
NCBI Protein Information
glycine cleavage system H protein, mitochondrial
UniProt Protein Name
Glycine cleavage system H protein, mitochondrial
UniProt Synonym Protein Names
Lipoic acid-containing protein
Protein Family
Glycine cleavage system H-like protein
UniProt Gene Name
GCSH [Similar Products]
UniProt Entry Name
GCSH_HUMAN
NCBI Summary for GCSH
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]
UniProt Comments for GCSH
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
Research Articles on GCSH
1. There is no detectable glycine cleavage enzyme activity in human skin fibroblasts.
Precautions
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