GCDH anticorps (AA 201-300) (Biotin)

N° du produit ABIN1700307

Cet anticorps anti-GCDH est un anticorps Lapin Polyclonal détectant GCDH dans WB, ELISA, IHC (p) et IHC (fro). Adapté pour Humain.

Aperçu rapide pour GCDH anticorps (AA 201-300) (Biotin) (ABIN1700307)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GCDH est conjugé à/à la Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Détail du produit anti-GCDH anticorps (Biotin)

Épitope: AA 201-300

Homologie: Human,Mouse,Rat

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human GCDH

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

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.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C for 12 months.

Date de péremption: 12 months

Détails sur GCDH

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

Autre désignation: GCDH

Sujet:

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.

ID gène: 2639