Article
Mitochondrial trifunctional protein defects: molecular basis and novel therapeutic approaches.
Current drug targets. Immune, endocrine and metabolic disorders - 1 Mar 2005
Angdisen J, Moore V D G, Cline J M, Payne R M, Ibdah J A
Abstract excerpt
Mitochondrial trifunctional protein (MTP) is a complex protein that catalyzes the last three steps of long chain fatty acid oxidation. MTP defects have emerged recently as important inborn errors of metabolism because of their clinical implications. These disorders are recessively inherited and display a spectrum of clinical phenotypes in affected children including hepatic dysfunction, cardiomyopathy,...
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