GCDH antibody (AA 201-300) (AbBy Fluor® 750)

Catalog No. ABIN5003282

This anti-GCDH antibody is a Rabbit Polyclonal antibody detecting GCDH in WB, IF (cc) and IF (p). Suitable for Human.

Quick Overview for GCDH antibody (AA 201-300) (AbBy Fluor® 750) (ABIN5003282)

Target: GCDH (Glutaryl-CoA Dehydrogenase (GCDH))

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GCDH antibody is conjugated to AbBy Fluor® 750

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

Product Details anti-GCDH Antibody (AbBy Fluor® 750)

Binding Specificity: AA 201-300

Predicted Reactivity: Human,Mouse,Rat

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GCDH

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 GCDH

Target: GCDH (Glutaryl-CoA Dehydrogenase (GCDH))

Alternative Name: GCDH

Background:

Synonyms: ACAD5, EC 1.3.99.7, GCD, Gcdh, GCDH_HUMAN, Glutaryl CoA dehydrogenase, mitochondrial, Glutaryl Coenzyme A dehydrogenase, Glutaryl-CoA dehydrogenase, mitochondrial, MS781.

Background: GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.

Gene ID: 2639