Key features and details
- Mouse monoclonal [1A12BC8] to HADH
- Suitable for: IHC-P, ICC/IF, IP, Flow Cyt, In-Cell ELISA
- Reacts with: Human
- Isotype: IgG1
Product nameAnti-HADH antibody [1A12BC8]
See all HADH primary antibodies
DescriptionMouse monoclonal [1A12BC8] to HADH
Tested applicationsSuitable for: IHC-P, ICC/IF, IP, Flow Cyt, In-Cell ELISAmore details
Species reactivityReacts with: Human
Tissue, cells or virus. This information is considered to be commercially sensitive.
- MRC5 fibroblasts; Human cerebellum tissue; Human liver mitochondria; HL-60 cells.
This antibody clone is manufactured by Abcam.
Product was previously marketed under the MitoSciences sub-brand.
This product was previously labelled as HADHSC
Storage instructionsShipped at 4°C. Store at +4°C. Do Not Freeze.
Storage bufferPreservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
Purification notesPurity near homogeneity as judged by SDS-PAGE (purity >95%). The antibody was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
Light chain typekappa
- Pathways and Processes
- Metabolic signaling pathways
- Lipid and lipoprotein metabolism
- Lipid metabolism
Our Abpromise guarantee covers the use of ab110284 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/250. Perform heat mediated antigen retrieval - 1 min pressure cook in 1mmol EDTA pH8.|
|ICC/IF||Use a concentration of 5 µg/ml.|
|IP||Use at an assay dependent concentration.|
|Flow Cyt||Use a concentration of 1 µg/ml.
ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
|In-Cell ELISA||Use a concentration of 2 µg/ml. (0.2 µg/well).|
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
MRC5 fibroblasts labeled with ab110284 at 5µg/ml and stained with Texas Red conjugated to goat anti-mouse secondary.
HADH immunocaptured from Human liver mitochondria using ab110284.
HL-60 cells were stained with 1 µg/ml ab110284 (blue) or an equal amount of an isotype control antibody (red) and analyzed by flow cytometry.
ab110284, at 1/250 dilution, staining HADH in formalin-fixed, paraffin-embedded Human cerebellum by Immunohistochemistry. HADH immunoactivity is most intense in neuronal cell bodies, most notably in the large Purkinje cells.
ab110284 has not yet been referenced specifically in any publications.