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

Produktnummer ABIN5003281

Der Kaninchen Polyklonal Anti-GCDH-Antikörper wurde für WB, IF (cc) und IF (p) validiert. Er ist geeignet, GCDH in Proben von Human zu detektieren.

Kurzübersicht für GCDH Antikörper (AA 201-300) (AbBy Fluor® 680) (ABIN5003281)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GCDH Antikörper ist konjugiert mit AbBy Fluor® 680

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

Produktdetails anti-GCDH Antikörper (AbBy Fluor® 680)

Bindungsspezifität: AA 201-300

Homologie: Human,Mouse,Rat

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GCDH

Isotyp: IgG

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu GCDH

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

Andere Bezeichnung: GCDH

Hintergrund:

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.

Gen-ID: 2639