GCSH antibody (AA 101-173) (Cy3)

Catalog No. ABIN1702123

This Rabbit Polyclonal antibody specifically detects GCSH in WB, IF (cc) and IF (p). It exhibits reactivity toward Human.

Quick Overview for GCSH antibody (AA 101-173) (Cy3) (ABIN1702123)

Target: GCSH (Glycine Cleavage System H Protein (GCSH))

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GCSH antibody is conjugated to Cy3

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Product Details anti-GCSH Antibody (Cy3)

Binding Specificity: AA 101-173

Predicted Reactivity: Human,Mouse,Rat,Horse

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GCSH

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for GCSH

Target: GCSH (Glycine Cleavage System H Protein (GCSH))

Alternative Name: GCSH

Background:

Synonyms: GCE, GCSH, GCSH_HUMAN, Glycine cleavage system H protein, Glycine cleavage system H protein mitochondrial, Glycine cleavage system protein H aminomethyl carrier, Glycine cleavage system protein H, Lipoic acid containing protein, mitochondrial, Mitochondrial glycine cleavage system H protein, NKH.

Background: GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3 % of the human genome.

Gene ID: 2653