Current Views of Fatty Acid Oxidation and Ketogenesis: From Organelles to Point Mutations

ISBN-13: 9781475772111 / Angielski / Miękka / 2013 / 414 str.

Patti A. Quant; Simon Eaton

Opis

In September 1991, Victor Zammit and I were in the Department of Biochemistry, the University of Cambridge, discussing ou r collaborative research project when we real ized thepotential value and need fora conference specifically concerned with fatty acid oxidation and ketogenesis. Thei dea, onceseeded, was indulged and flourished into the first "Fatty Acid Oxidation & Ketogenesis (FAOx&K) Conference"t hatwas held in the Department at Eastertime, 1992. Itw as attended byc olleagues mainly from the U. K., France and Spain. From these modest beginnings atradition for holding a conference every second yearh asgrown and this Book results from the 4th International FAOx&K Conference that was held in London at the Institute of Child Health & Great Ormond Street Hospital forChildren NHSTrust, University College London Medical School in the new Conference Suite and was attended by colleagues from over twenty c ountries and five different c ontinents. I would like to thank allm y colleagues who havec ontributed to the conferences and, mos t importantly, to this Book. The first two c onferences were held in the University of Cambridge and were orga nized entirely by me but Simon Eaton, who came to work in London with me in Febru ary 1997, b ecame Conference Secretary andco organized the last two meetings. His contribution tot he conferences has been invaluable and w ithout hisdedicated help and effort neither the latermeetings n ort his Book would haveb een possible.

Spis treści

Preface & Dedication; P.A. Quant, S. Eaton. Historical overview. Cambridge, colleagues, carnitine and ketogenesis .......; P.K. Tubbs. Carnitine and carnitine acyltransferases. 1. Biogenesis of the rat liver mitochondrial carnitine palmitoyltransferase I; I. Cohen, et al. 2. Subcellular distribution of mitochondrial carnitine palmitoyltransferase I in rat liver: Evidence for a distinctive N-terminal structure of the microsomal but not the peroxisomal enzyme; V.A. Zammit,et al. 3. Topology of hepatic mitochondrial carnitine palmitoyltransferase I; K. Kashfi, G.A. Cook. 4. Possible involvement of cytoskeletal components in the control of hepatic carnitine palmitoyltransferase I activity; G. Velasco, et al. 5. Regulation of beta-oxidation and CPT I activity by 3-thia fatty acids in cultured rat hepatocytes; J. Sleboda, J. Bremer. 6. Carnitine acyltransferases and associated transport systems in the endoplasmic reticulum; N.M. Broadway, et al. 7. Reciprocal enzymatic interference of carnitine palmitoyl transferase I and glycerol-3-phosphate acyltransferase in purified liver mitochondria; F. Beauseigneur, et al. 8. Control of human muscle carnitine palmitoyltransferase I gene transcription by peroxisome proliferator-activated receptor ; C. Mascaró, et al. 9. Kinetic investigation of carnitine palmitoyltransferases in homogenates of human skeletal muscle using L-aminocarnitine and malonyl-CoA; K. Hertel, et al. 10. Processing of carnitine octanoyltransferase pre-mRNAs by cis- and trans-splicing; C. Caudevilla, et al. 11. Selective modulation of carnitine long-chain acyltransferase activities: kinetics, inhibitors andactive sites of COT and CPT II; R.R. Ramsay, R.D. Gandour. 12. Confocal laser scanning microscopy of human skin fibroblasts showing transient expression of a green fluorescent carnitine palmitoyltransferase 1 fusion protein; F.R. van der Leij, et al. 13. Carnitine biosynthesis: purification of gamma-butyrobetaine from rat liver; F. Vaz, et al. Mitochondrial fatty acid oxidation and ketogenesis. 14. Hypolipidemic 3-thia fatty acids: Fatty acid oxidation and ketogenesis in rat liver under proliferation of mitochondria and peroxisomes; R.K. Berge, et al. 15. Molecular mechanisms of fatty acid &bgr;-oxidation enzyme catalysis; S.-Y. Yang, X.-Y. He. 16. Control of mitochondrial &bgr;-oxidation at the level of [NAD+]/[NADH] and CoA acylation; S. Eaton, et al. 17. Production and export of acyl-carnitine esters by neonatal rat hepatocytes; S. Eaton, et al. 18. Tissue specific differences in intramitochondrial control of &bgr;-oxidation; S. Eaton, K. Bartlett. 19. Endotoxin-induced changes in very-low-density lipoprotein and myocardial utilisation of triacylglycerol from abnormal VLDL in the rat; D.G. Hole, et al. 20. Effect of valproic acid on the expression of acyl-CoA dehydrogenases in various tissues; M. Nagao, et al. 21. Formation of a human `electron transferring flavoprotein': medium chain acyl CoA dehydrogenase complex - preliminary evidence from crosslinking studies; A. Parker, P.C. Engel. 22. Cloning and regulation of peroxisome proliferator-induced acyl-CoA thioestereases from mouse liver; M. Hunt, et al. 23. Metabolic effects of 3-thia fatty acids in cancer cells; K.J. Tronstad,