Not to be confused with microsomal triglyceride transfer protein.
Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, LCHAD deficiency
Mitochondrial trifunctional protein (MTP) is a protein attached to the inner mitochondrial membrane which catalyzes three out of the four steps in beta oxidation. MTP is a hetero-octamer composed of four alpha and four beta subunits:
HADHA
HADHB
The three functions are 2-enoyl coenzyme A (CoA) hydratase, long-chain 3-hydroxy acyl-coenzyme A dehydrogenase and long-chain 3-ketoacyl CoA thiolase.[1]
^"Long-Chain Acyl CoA Dehydrogenase Deficiency: eMedicine Pediatrics: Genetics and Metabolic Disease". Retrieved 2009-07-11.
and 19 Related for: Mitochondrial trifunctional protein information
Mitochondrialtrifunctionalprotein (MTP) is a protein attached to the inner mitochondrial membrane which catalyzes three out of the four steps in beta...
Mitochondrialtrifunctionalprotein deficiency (MTP deficiency or MTPD) is an autosomal recessive fatty acid oxidation disorder that prevents the body...
encodes the alpha subunit of the mitochondrialtrifunctionalprotein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty...
is encoded by the HADHB gene. HADHB is a subunit of the mitochondrialtrifunctionalprotein and has thiolase activity. The HADHB gene is located on chromosome...
primarily facilitated by the mitochondrialtrifunctionalprotein, an enzyme complex associated with the inner mitochondrial membrane, although very long...
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substrates for methionine, thymidylate, and de novo purine syntheses. The trifunctional enzymatic activities are conferred by two major domains, an aminoterminal...
counterpart. Also, it has been found to be a subunit of the peroxisomal trifunctional enzyme (pTFE) and contributes only to minor cleavages of the fatty acid...
in the form of sub-lingual tablets, patches, or as an aerosol spray. Trifunctional polyether polyols are produced from glycerol and propylene oxide. Oxidation...
involved in THF synthesis in mitochondria. In contrast to MTHFD1 that has trifunctional methylenetetrahydrofolate dehydrogenase, methenyltetrahydrofolate cyclohydrolase...
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