GCDH Antikörper (AA 201-300) (Biotin)

Produktnummer ABIN1700307

Dieses Anti-GCDH-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von GCDH in WB, ELISA, IHC (p) und IHC (fro). Geeignet für Human.

Kurzübersicht für GCDH Antikörper (AA 201-300) (Biotin) (ABIN1700307)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GCDH Antikörper ist konjugiert mit Biotin

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

Produktdetails anti-GCDH Antikörper (Biotin)

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: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

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 for 12 months.

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