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.
应用类型
Western blotting （0.5-4 ug/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.
免疫原
Synthetic peptide surrounding amino acid 750 of human TFP1
来源宿主
兔
反应性
经检测验证，可与人、小鼠、大鼠种属来源的TFP1蛋白发生免疫反应.
保存建议
提供的TFP1抗体产品为100 ug 无色溶液，其浓度为0.5 mg/ml，溶解于包含有30%甘油， 0.5% BSA和0.01%硫柳汞的PBS缓冲液中.请将该TFP1抗体产品置于-20 ℃冷冻保存，建议分装使用以避免反复冻融.
