Mitochondrial trifunctional protein
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:
The three functions are
long-chain 3-ketoacyl CoA thiolase.[1]
Association with the electron transport chain
Fatty acid beta-oxidation (FAO) and
channel substrates between the two enzymes.[2]
This is especially interesting, because up until then it was unknown exactly how MTP was associated with the inner mitochondrial membrane, and this discovery may provide the explanation.
Hormonal influences
Recent research has revealed that MTP can be affected by various
thyroid hormone stimulates mitochondrial metabolism in a pathway mediated by MTP.[3] Zhou et al. (2012) used 2D gel electrophoresis and mass spectrometry to identify MTP as one of the proteins that interacts with ER alpha, a receptor triggered by estrogen.[4]
Cardiolipin remodeling
In 2009, Taylor et al. identified a human mitochondrial protein,
C-terminal end of MTP, linking MTP to the remodeling of cardiolipin from monolysocardiolipin.[5] Although MLCL AT-1 and MTP are different proteins, in 2012 the same lab discovered that MTP did indeed have cardiolipin remodeling capabilities.[6] This suggests a possible link between mitochondrial membrane cardiolipin content and beta oxidation
.
Clinical significance
Disorders associated MTP are:
References
External links
- mitochondrial+trifunctional+protein+TP at the U.S. National Library of Medicine Medical Subject Headings (MeSH)