GCSH antibody (AA 101-173) (Alexa Fluor 594)

Catalog No. ABIN2813654

Product Details anti-GCSH Antibody (Alexa Fluor 594)

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

Binding Specificity: AA 101-173

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GCSH antibody is conjugated to Alexa Fluor 594

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

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 (GCSH Products)

Synonyms: GCE antibody, NKH antibody, GCSH antibody, H-PROTEIN antibody, 1100001L02Rik antibody, 5730591C18Rik antibody, wu:fb14d10 antibody, wu:fw84b08 antibody, zgc:112535 antibody, BA5228 antibody, gce antibody, gcsh antibody, nkh antibody, wu:fd56h06 antibody, zgc:110557 antibody, zgc:92732 antibody, glycine cleavage system protein H antibody, glycine cleavage system protein H (aminomethyl carrier) antibody, glycine cleavage system lipoyl carrier protein GcvH antibody, glycine cleavage system H protein antibody, glycine cleavage system protein H (aminomethyl carrier), a antibody, glycine cleavage system carrier of aminomethyl moiety GcvH antibody, leucine rich repeat containing 34 antibody, glycine cleavage system protein H (aminomethyl carrier) L homeolog antibody, glycine cleavage system protein H (aminomethyl carrier), b antibody, GCSH antibody, Gcsh antibody, gcvH-2 antibody, gcvH antibody, gcsha antibody, LRRC34 antibody, gcsh.L antibody, gcshb antibody

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