TFP1 Antibody

Antibody Target: TFP1

Host: Rabbit

Antibody Type: Polyclonal

Isotype: Rabbit IgG

Antibody Form: Affinity Purified Antibody

Immunogen: Synthetic peptide surrounding amino acid 750 of human TFP1

Accession#: P40939

Gene ID: 3030

Appearance: Colorless liquid

Concentration: 0.5 mg/ml

Formulation: 100 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

Species Reactivity: hu/ms/rt

Application: WB

Application & Usage: 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.

Storage Temp.: -20 °C

Shipping : gel pack

Shelf Life: 12 months

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

Brief Background Descriptions: 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.