anti-TFP1/HADHA Antibody

TFP1

Mitochondrial Trifunctional Protein

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.

Rabbit

Polyclonal

Rabbit IgG

Human, mouse, rat

Synthetic peptide surrounding amino acid 750 of human TFP1

0.5 mg/ml

Liquid

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.

Affinity purified

Western blot

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.

Shelf time: 12 months in temp.: -20 deg.C

gel pack

The antibody solution should be gently mixed before use.

This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided.

If you buy Antibodies supplied by Biovision they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.

anticorps