Open AccessMitochondrial β‐oxidation
Author(s) -
Bartlett Kim,
Eaton Simon
Publication year - 2004
Publication title -
european journal of biochemistry
Language(s) - English
Resource type - Journals
eISSN - 1432-1033
pISSN - 0014-2956
DOI - 10.1046/j.1432-1033.2003.03947.x
Subject(s) - mitochondrial matrix , inner mitochondrial membrane , flux (metallurgy) , beta oxidation , mitochondrion , chemistry , carnitine , enzyme , biochemistry , microbiology and biotechnology , transferase , biology , cytosol , organic chemistry
Mitochondrial β‐oxidation is a complex pathway involving, in the case of saturated straight chain fatty acids of even carbon number, at least 16 proteins which are organized into two functional subdomains; one associated with the inner face of the inner mitochondrial membrane and the other in the matrix. Overall, the pathway is subject to intramitochondrial control at multiple sites. However, at least in the liver, carnitine palmitoyl transferase I exerts approximately 80% of control over pathway flux under normal conditions. Clearly, when one or more enzyme activities are attenuated because of a mutation, the major site of flux control will change.