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.
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Pack Size | 100ul, 1ml, 20ul |
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Applications | ELISA, IF/ICC, IHC-P, IP, WB |
Host Species | Rabbit |
Ig Isotype | IgG |
Antibody Type | Monoclonal Antibody |
Organism Species | Human |
Concentration | – |
Product Synonyms | HADHA |
Observed Mol Wt | 83kDa |
Alternative Names | GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA |
Immunogen (Antigen) | Recombinant protein |
Format | Liquid |
Buffer Formulation | 0.05% BSA, 50% glycerol, PBS with 0.05% proclin300, pH7.3 |
Reactivity | Human, Mouse, Rat |
Uniprot ID | 3030 |
Gene ID | P40939 |
Purification | Antigen-specific affinity chromatography followed by Protein A affinity chromatography |
Usage | For Research Use Only. Not for diagnostics or human use. |
Shelf Life | 12 months at time of shipping |
Shipping | Shipped in Dry Ice at -20 Degree Celsius |
Storage | Store at -20 Degree Celsius. It is recommended to aliquot and store to avoid repeated freeze-thaw as it affects the stability of the antibody. |
Research Areas | Cancer, Cell Biology, Immunology |
KD/KO Validated | KD Validated |
Disclaimer | The data indicated herein are as indicated and validated in our laboratory. These reagents are for research use only and not for in-vitro diagnostics or human use. |
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