HADH polyclonal, anti-human, mouse, rat
€388.00
In stock
SKU
BS6128
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.
Alternative Name:
HCDH, , Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial, Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase, Short-chain 3-hydroxyacyl-CoA dehydrogenase,
Application Dilution: WB: 1:500~1:2000, IHC: 1:50~1:200
Specificity: HADH polyclonal antibody detects endogenous levels of HADH protein.
Immunogen:
Recombinant full length Human HADH.
MW: ~ 34 kDa
Swis Prot.: Q16836
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).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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.
Alternative Name:
HCDH, , Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial, Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase, Short-chain 3-hydroxyacyl-CoA dehydrogenase,
Application Dilution: WB: 1:500~1:2000, IHC: 1:50~1:200
Specificity: HADH polyclonal antibody detects endogenous levels of HADH protein.
Immunogen:
Recombinant full length Human HADH.
MW: ~ 34 kDa
Swis Prot.: Q16836
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).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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