Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term.
Storage bufferConstituent: PBS
Concentration information loading...
PurityImmunogen affinity purified
- Pathways and Processes
- Metabolic signaling pathways
- Lipid and lipoprotein metabolism
- Lipid metabolism
Our Abpromise guarantee covers the use of ab37673 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/2000. Predicted molecular weight: 35 kDa.|
FunctionPlays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
Tissue specificityExpressed in liver, kidney, pancreas, heart and skeletal muscle.
PathwayLipid metabolism; fatty acid beta-oxidation.
Involvement in diseaseDefects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency) [MIM:231530]. 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) [MIM:609975]; 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.
Sequence similaritiesBelongs to the 3-hydroxyacyl-CoA dehydrogenase family.
Cellular localizationMitochondrion matrix.
- Information by UniProt
- 3 hydroxyacyl Coenzyme A dehydrogenase antibody
- HAD antibody
- HADH antibody
Anti-HADHSC antibody - Azide free (ab37673) at 1/2000 dilution
Goat anti-IgY-HRP. at 1/1000 dilution
Predicted band size: 35 kDa
Observed band size: 80 kDa why is the actual band size different from the predicted?
E. coli-derived fusion protein as test antigen.
This product has been referenced in:
- Fukushima A et al. Acetylation contributes to hypertrophy-caused maturational delay of cardiac energy metabolism. JCI Insight 3:N/A (2018). Read more (PubMed: 29769443) »
- Stocks B et al. Skeletal Muscle Fibre-Specific Knockout of p53 Does Not Reduce Mitochondrial Content or Enzyme Activity. Front Physiol 8:941 (2017). Read more (PubMed: 29255419) »