HADH, Polyclonal Antibody

HAD; HADHSC; SCHAD; Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; HCDH; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Recognizes endogenous levels of HADH protein.

The antibody was purified by immunogen affinity chromatography.

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Small volumes of anti-HADH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Rabbit polyclonal antibody to HADH

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1/500 - 1/2000; IHC: 1/50 - 1/200

Western blot analysis of HADH expression in Jurkat (A), HT29 (B), HL60 (C) whole cell lysates.

Immunohistochemical analysis of HADH staining in rat liver formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

36,051 Da

hydroxyacyl-CoA dehydrogenase

hydroxyacyl-coenzyme A dehydrogenase, mitochondrial

Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial

HAD; HADHSC; SCHAD; HCDH  [Similar Products]

HCDH_HUMAN

This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene of this gene on chromosome 15. [provided by RefSeq, May 2010]

HADHSC: 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. HHF is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. HHF4 should be easily recognizable by analysis of acylcarnitine species and that this disorder responds well to treatment with diazoxide. It provides the first 'experiment of nature' that links impaired fatty acid oxidation to hyperinsulinism and that provides support for the concept that a lipid signaling pathway is implicated in the control of insulin secretion. Belongs to the 3-hydroxyacyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Carbohydrate Metabolism - butanoate; Lipid Metabolism - fatty acid; Lipid Metabolism - fatty acid elongation in mitochondria; EC 1.1.1.35; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Amino Acid Metabolism - lysine degradation; Oxidoreductase; Amino Acid Metabolism - tryptophan

Chromosomal Location of Human Ortholog: 4q22-q26

Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrial matrix; mitochondrion; nucleoplasm

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity

Biological Process: fatty acid beta-oxidation; negative regulation of insulin secretion; response to activity; response to drug; response to insulin stimulus

Disease: 3-hydroxyacyl-coa Dehydrogenase Deficiency; Hyperinsulinemic Hypoglycemia, Familial, 4

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