TFP1/HADHA Antibody

TFP1/HADHA Antibodyis available at Gentaur for Next week Delivery.

Description: 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.

Immunogen Sequence:Synthetic peptide surrounding amino acid 750 of human TFP1

Target Alternative Name:Mitochondrial Trifunctional Protein

Species Reactivities:Human, mouse, rat

Tag Line:/

Formulation:30 µg (0.5 mg/ml) affinity purified rabbit polyclonal antibody in 1X phosphate-buffered saline (PBS) containing 30% glycerol, 0.5% BSA, and 0.01% thimerosal.

Purification: Affinity purified

Application:Western blot

Positive Control:/

Application And Usages: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.

Handling:The antibody solution should be gently mixed before use.