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HADH polyclonal Antibody | BS6128

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BW-BS6128
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NULL444.00 - NULL678.00

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:

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