TFP1/HADHA Antibody
Mitochondrial 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.
| Supplier | Creative Biogene |
|---|---|
| Product # | PBCA1058 |
| Pricing | Inquiry |
| Isotype | Rabbit IgG |
| Research Area | Antibody |
| Antibody Target | TFP1 |
| Antibody Type | Polyclonal |
| Application | WB |
| Formulation | 0.5 mg/ml of antibody in 1X PBS containing 30% glycerol, 0.5% BSA, and 0.01% thimerosal |
| Appearance | Liquid |
| Host | Rabbit |
| Species Reactivity | Human, Mouse, Rat |
| Usage Statement | Our products are intended for research use only and are not to be used for other purposes, including but are not limited to in vitro diagnostic uses, ex vivo or in vivo therapeutic uses. |