Mitochondrial trifunctional protein defects: clinical implications and therapeutic approaches.

doi:10.1016/j.addr.2008.04.014

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Article

Mitochondrial trifunctional protein defects: clinical implications and therapeutic approaches.

Advanced drug delivery reviews - 1 Jan 2000

Rector R Scott, Payne R Mark, Ibdah Jamal A

Abstract excerpt

The mitochondrial trifunctional protein (MTP) is a heterotrimeric protein that consists of four alpha-subunits and four beta-subunits and catalyzes three of the four chain-shortening reactions in the mitochondrial beta-oxidation of long-chain fatty acids. Families with recessively inherited MTP defects display a spectrum of maternal and fetal phenotypes. Current management of patients with MTP defects include...

Topics

Diet
Fatty Acids
Genetic Therapy
Humans
Mitochondrial Diseases
Mitochondrial Trifunctional Protein
Multienzyme Complexes
Oxidation-Reduction
Phenotype

Source

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Identifiers

PMID: 18652860
DOI: 10.1016/j.addr.2008.04.014
PMCID: PMC2848452

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