Description
HADH polyclonal Antibody | BS6128 | Gentaur UK, US & Europe Distribution
Host: Rabbit
Reactivity: Human,Mouse,Rat
Application: WB IHC
Application Range: WB 1:500 - 1:2000 IHC 1:50 - 1:200
Background: Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.Defects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency) . HADH deficiency is a metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death. Defects in HADH are the cause of familial hyperinsulinemic hypoglycemia type 4 (HHF4) ; also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or congenital hyperinsulinism.
Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity: HADH polyclonal Antibody detects endogenous levels of HADH protein.
Molecular Weight: ~ 34 kDa
Note: For research use only, not for use in diagnostic procedure.
Alternative Names: HCDH; ; Hydroxyacyl-coenzyme A dehydrogenase; mitochondrial ; ; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase ; ; Short-chain 3-hydroxyacyl-CoA dehydrogenase;
Immunogen: Recombinant full length Human HADH.
Conjugate: Unconjugated
Modification: Unmodification
Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Pathway: