GCDH anticorps (AA 201-300) (AbBy Fluor® 680)

N° du produit ABIN5003281

L’anticorps Lapin Polyclonal anti-GCDH a été validé pour WB, IF (cc) et IF (p). Il convient pour détecter GCDH dans des échantillons de Humain.

Aperçu rapide pour GCDH anticorps (AA 201-300) (AbBy Fluor® 680) (ABIN5003281)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GCDH est conjugé à/à la AbBy Fluor® 680

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

Détail du produit anti-GCDH anticorps (AbBy Fluor® 680)

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

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. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

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