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Brief Description & Uses Contains 30 or 60 dipsticks and necessary components to quantify the amount of the Mitochondrial Trifunctional Protein (MTP) from human sample material (rodent samples are not immuno-reactive). The kit includes sufficient materials to generate a standard curve and prepare twenty samples for analysis. MTP 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. MTP is 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.4.2.1.17) and the dehydrogenation of 3-hydroxyacyl CoA (LCHAD - E.C.1.1.1.211). The β-subunit, a 47 kDa enzyme, performs the third function - the thiolytic cleavage of 3-ketoacyl-CoA substrates (E.C.2.3.1.16). Inherited deficiency of MTP is a recessive genetic disease which occurs in approximately 1:38,000 newborns in the US. Deficiency of LCHAD (the dehydrogenase part of MTP) 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 MTP 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%. Specifications
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Have you published research using this product? Let us know and earn a $100.00 discount on your next purchase. References for Mitochondrial Trifunctional Protein (MTP)
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Images The above results show the accuracy and precision of the MTP dipstick kit (MS741) in measuring reduced amounts of MTP when a sample is diluted. HepG2 cell extract was diluted to 12.5% of the undiluted sample in 50% increments. Graphical analysis was performed using values interpolated from a standard curve. All analysis was done with the MS1000 dipstick reader and GraphPad software.
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