HADHA Mouse Monoclonal Antibody (Biotin conjugated) [Clone ID: OTI7B3]
Other products for "HADHA"
Specifications
Product Data | |
Clone Name | OTI7B3 |
Applications | WB |
Recommended Dilution | WB 1:500 |
Reactivities | Human, Mouse, Rat |
Host | Mouse |
Isotype | IgG1 |
Clonality | Monoclonal |
Immunogen | Human recombinant protein fragment corresponding to amino acids 106-325 of human HADHA (NP_000173) produced in E.coli. |
Formulation | PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide. |
Concentration | 0.5 mg/ml |
Purification | Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G) |
Conjugation | Biotin |
Storage | Store at -20°C as received. |
Stability | Stable for 12 months from date of receipt. |
Predicted Protein Size | 83 kDa |
Gene Name | hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit |
Database Link | |
Background | This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008] |
Synonyms | ECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA |
Reference Data | |
Protein Families | Druggable Genome |
Protein Pathways | beta-Alanine metabolism, Biosynthesis of unsaturated fatty acids, Butanoate metabolism, Fatty acid elongation in mitochondria, Fatty acid metabolism, Limonene and pinene degradation, Lysine degradation, Metabolic pathways, Propanoate metabolism, Tryptophan metabolism, Valine, leucine and isoleucine degradation |
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