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Mouse Monoclonal antibody anti Mitochondrial Trifunctional Protein (TFP) monoclonal antibody (Sku# 459180) is reactive against human protein, and is validated in Western blotting, and Immunocapture applications. TFP is part of the mitochondrial fatty acid beta-oxidation pathway. It is located in the inner mitochondrial membrane and it catalyzes three out of the four steps in the beta-oxidation cycle with a specific affinity for long chain substrates. TFP is a hetero-octamer composed of four α and four β-subunits. The α-subunit is a 79 kDa enzyme which has two enzymatic functions, the hydration of enoyl-CoA (E.C.126.96.36.199) and the dehydrogenation of 3-hydroxyacyl CoA (LCHAD - E.C.188.8.131.52). The β-subunit, a 47 kDa enzyme, performs the third function - the thiolytic cleavage of 3-ketoacyl-CoA substrates (E.C.184.108.40.206). Inherited deficiency of TFP is a recessive genetic disease which occurs in approximately 1:38,000 newborns in the US. Deficiency of LCHAD (the dehydrogenase part of TFP) has also been found in children of women who develop HELLP syndrome (1:1,000 pregnancies) and AFLP syndrome (1:13,000 pregnancies), both of which are life-threatening obstetric conditions. Furthermore, heterozygous individuals for TFP defects are more susceptible to nonalcoholic hepatic steatosis, or nonalcoholic fatty liver disease (NAFLD), the most common form of liver disease in the US with a recorded prevalence as high as 39%.
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