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.
應用類型
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
來源宿主
兔
反應性
經檢測驗證，可與人、小鼠、大鼠種屬來源的TFP1蛋白發生免疫反應.
保存建議
提供的TFP1抗體產品為100 ug 無色溶液，其濃度為0.5 mg/ml，溶解于包含有30%甘油， 0.5% BSA和0.01%硫柳汞的PBS緩沖液中.請將該TFP1抗體產品置于-20 ℃冷凍保存，建議分裝使用以避免反復凍融.
