MS740 Rapid Microplate Assay for Human
Mitochondrial Trifunctional Protein
$325.00
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Brief Description & Uses
This rapid quantity microplate kit is used to determine the assembled enzyme quantity of Mitochondrial Trifunctional Protein (MTP) in a human sample with speed and simplicity. The MTP enzyme is immunocaptured within the wells of the microplate and the amount of enzyme is determined with an ELISA sandwich assay coupled to an alkaline phosphatase reaction. The reaction takes place in a time dependent manner proportional to the amount of enzyme captured in the wells. This kit includes all reagents necessary for 96 well measurements, allowing for a stream-lined assay.
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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Product:
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Rapid Microplate Assay
for Human Mitochondrial Trifunctional Protein (MTP)
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Catalog #:
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MS740
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Purity:
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Near homogeneity as judged by SDS-PAGE. The antibodies were produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
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Specificity:
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Human
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Applications:
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MTP expression measurement
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Storage:
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Buffers, detergent, and microplate should be stored at 4°C. Development reagent
must be stored at -20°C
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Country of Origin:
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USA
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References for Mitochondrial Trifunctional Protein (MTP)
There are no references currently posted for this enzyme.
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