tailieunhanh - Báo cáo khóa học: Mitochondrialb-oxidation

Mitochondrialb-oxidation is a complex pathway involving, in the case of saturated straight chain fatty acids of even carbonnumber, at least 16proteinswhichare 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 intramito-chondrial 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 | Eur. J. Biochem. 271 462-469 2004 FEBS 2004 doi MINIREVIEW Mitochondrial b-oxidation Kim Bartlett1 and Simon Eaton2 1 Department of Child Health Sir James Spence Institute of Child Health University of Newcastle upon Tyne Royal Victoria Infirmary Newcastle upon Tyne 2Surgery Unit and Biochemistry Endocrinology and Metabolism Unit Institute of Child Health University College London UK Mitochondrial b-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 intramito- chondrial 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. Introduction The b-oxidation of long-chain fatty acids is central to the provision of energy for the organism and is of particular importance for cardiac and skeletal muscle. However a number of other tissues primarily the liver but also the kidney small intestine and white adipose tissue can utilize the products of b-oxidation for the formation of ketone bodies which can in turn be utilized for energy by other tissues. The relationship of fat oxidation with the utilization of carbohydrate as a source of energy is complex and depends upon tissue nutritional state exercise development and a variety of other influences such as infection and other pathological states. A full description of the regulatory mechanisms involved is beyond the scope of the present review and the interested reader is referred to recent treatments of the subject 1-5 . In the present review we concentrate on the response to stress and fasting