Application
Western blot analysis of TFP-1 expression with lysate from Jurkat cells (Lane 1,2), 3T3 cells (Lane 3) and rat kidney (Lane 4).| Product Name | HADHA antibody |
|---|---|
| Antibody Type | Primary Antibodies |
| Product description | Rabbit polyclonal antibody to HADHAMitochondrial Trifunctional Protein (TFP) is a multienzyme complex of the beta-oxidation cycle. TFP deficiency is a clinically heterogeneous disorder with phenotypes of different severity. The spectrum of diseases range from severe neonatal/infantile cardiomyopathy and early death to mild chronic progressive sensorimotor poly-neuropathy with episodic rhabdomyolysis. Human TFP is an octomer composed of four alpha-subunits and four beta-subunits. Mutations in either subunits may result in general TFP deficiency with reduced activity of all enzymes. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WB |
| Western blotting (0.5-4 μg/ml). However, the optimal concentrations should be determined individually. The antibody recognizes 84 kDa TFP1 from samples of human, mouse and rat origins. Reactivity to other species has not been tested.: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 0.5mg/ml |
| Purification | Affinity purified |
| Gene Synonyms | Mitochondrial Trifunctional Protein |
|---|---|
| Target | TFP1 |
| Tissue Specificity | This antibody is specific for TFP1. |
Application
Western blot analysis of TFP-1 expression with lysate from Jurkat cells (Lane 1,2), 3T3 cells (Lane 3) and rat kidney (Lane 4).| Application Notes | Western blotting (0.5-4 μg/ml). However, the optimal concentrations should be determined individually. The antibody recognizes 84 kDa TFP1 from samples of human, mouse and rat origins. Reactivity to other species has not been tested.: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | The antibody solution should be gently mixed before use. Stored at -20 °C for future use. |
| Storage Buffer | 1X PBS (pH 7.2) containing 30% glycerol, 0.5% BSA, and 0.01% thimerosal |
