Current views of fatty acid oxidation and ketogenesis: from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England]
Saved in:
| Format: | Book |
|---|---|
| Language: | English |
| Published: |
New York [u.a.]
Kluwer
1999
|
| Series: | Advances in experimental medicine and biology
466 |
| Subjects: | |
| Online Access: | Inhaltsverzeichnis |
| Physical Description: | XVII, 414 S. Ill., graph. Darst. |
| ISBN: | 0306462001 |
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1
CONTENTS
i
f
j
i
Cambridge, Colleagues, Carnitine and Ketogenesis xv
Philip K. Tubbs
1. Biogenesis of the Rat Liver Mitochondrial Carnitine
! Palmitoyltransferase I 1
Isabelle Cohen, Jean Girard, and Carina Prip Buus
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 17
Fiona Fraser, Clark G. Corstorphine, and Victor A. Zammit
3. Topology of Hepatic Mitochondrial Carnitine Palmitoyltransferase I 27
Khosrow Kashfi and George A. Cook
i
4. Possible Involvement of Cytoskeletal Components in the Control
of Hepatic Carnitine Palmitoyltransferase I Activity 43
i Guillermo Velasco, Math J. H. Geelen, Teresa Gomez del Pulgar,
and Manuel Guzman
5. Effects of 3 Thia Fatty Acids on P Oxidation and Carnitine
Palmitoyltransferase I Activity in Cultured Rat Hepatocytes 53
Jowita Sleboda and Jon Bremer
6. Carnitine Acyltransferases and Associated Transport Processes
in the Endoplasmic Reticulum: Missing Links in the VLDL
Story? 59
Neil M. Broadway, Jason M. Gooding, and E. David Saggerson
ix
x Contents
7. Reciprocal Enzymatic Interference of Carnitine Palmitoyltransferase I
and Glycerol 3 Phosphate Acyltransferase in Purified Liver
Mitochondria 69
Frederic Beauseigneur, Marcelline Tsoko, Joseph Gresti,
and Pierre Clouet
8. Characterization of a Response Element for Peroxisomal Proliferator t
Activated Receptor (PPRE) in Human Muscle Type Carnitine
Palmitoyltransferase I 79
Cristina Mascaro, Elena Acosta, Jose A. Ortiz,
Joan Carles Rodriguez, Pedro F. Marrero, Fausto G. Hegardt,
and Diego Haro
9. Kinetic Investigation of Carnitine Palmitoyltransferases in Homogenates
of Human Skeletal Muscle Using L Amino Carnitine and
Malonyl CoA 87
Kathrin Hertel, Frank Norbert Gellerich, Werner Hein,
and Stephan Zierz
10. Processing of Carnitine Octanoyltransferase Pre mRNAs by Cis and
7rans Splicing 95 ,
Concha Caudevilla, Dolors Serra, Angel Miliar,
Carles Codony, Guillermina Asins, Montserrat Bach,
and Fausto G. Hegardt
t
11. Selective Modulation of Carnitine Long Chain Acyltransferase
Activities: Kinetics, Inhibitors, and Active Sites of COT
and CPT II 103
Rona R. Ramsay and Richard D. Gandour
12. Confocal Laser Scanning Microscopy of Human Skin Fibroblasts
Showing Transient Expression of a Green Fluorescent Carnitine *
Palmitoyltransferase 1 Fusion Protein Ill
F. R. van der Leij, H. Roelofsen, K. E. Niezen Koning,
E. A. A. Nollen, and J. R. G. Kuipers
13. Carnitine Biosynthesis: Purification of y Butyrobetaine Hydroxylase from
Rat Liver 117
Frederic M. Vaz, Sandy van Gool, Rob Ofman, Lodewijk Ulst, and
Ronald J. A. Wanders
14. Hypolipidemic 3 Thia Fatty Acids: Fatty Acid Oxidation and
Ketogenesis in Rat Liver under Proliferation of Mitochondria and
Peroxisomes 125
Rolf K. Berge, Lise Madsen, and Hege Vaagenes
§
15. Molecular Mechanisms of Fatty Acid (3 Oxidation Enzyme Catalysis 133 |
Song Yu Yang and Xue Ying He I
Contents xj
16. Control of Mitochondrial (3 Oxidation at the Levels of [NAD+]/
[NADH] and CoA Acylation 145
Simon Eaton, Bruce Middleton, H. Stanley A. Sherratt,
Mori Pourfarzam, Patti A. Quant, and Kim Bartlett
17. Production and Export of Acyl Carnitine Esters by Neonatal Rat
Hepatocytes 155
Simon Eaton, Kim Bartlett, Mori Pourfarzam, Michele A. Markley,
Karen J. New, and Patti A. Quant
18. Tissue Specific Differences in Intramitochondrial Control of
P Oxidation 161
Simon Eaton and Kim Bartlett
19. Endotoxin Induced Changes in Very Low Density Lipoprotein and
Myocardial Utilisation of Triacylglycerol from Abnormal
VLDL in the Rat 169
David G. Hole, Mark J. Bennett, and Rhys D. Evans
20. Effect of Valproic Acid on the Expression of Acyl CoA
Dehydrogenases in Various Tissues 177
Masayoshi Nagao, Masahiro Kibayashi, and Shunzo Chiba
21. Formation of a Human Electron Transferring Flavoprotein :
Medium Chain Acyl Coenzyme A Dehydrogenase Complex,
Preliminary Evidence from Crosslinking Studies 191
Antony Parker and Paul C. Engel
22. Cloning and Regulation of Peroxisome Proliferator Induced Acyl
CoA Thioesterases from Mouse Liver 195
M. Hunt, P. J. G. Lindquist, S. Nousiainen, T. L. T. Svensson,
U. Diczfalusy, and S. E. H. Alexson
23. Metabolic Effects of 3 Thia Fatty Acid in Cancer Cells 201
Karl Johan Tronstad, Kjetil Berge, Rolf Bjerkvig, Torgeir Flatmark,
and Rolf K. Berge
24. Poorly Oxidizable Fatty Acid Analogues Inhibit the Proliferation of
Cancer Cells in Culture 205
Kjetil Berge, Karl Johan Tronstad, Abdi Dezfuli F., T. Ranheim,
R. Mahesparan, Rolf Bjerkvig, and Rolf K. Berge
25. The Role of PPARa as a Lipostat Transcription Factor 211
F. Djouadi, C. J. Weinheimer, and D. P. Kelly
26. The Hypolipidaemic Effect of EPA is Potentiated by 2 and
3 Methylation 221
Hege Vaagenes, Lise Madsen, Endre Dyroy, Livar Frayland,
Nina Willumsen, and Rolf K. Berge
xii Contents
27. Is It Time to Reconsider the Role of CPT I in Control of Hepatic
Ketogenesis? 227
Karen J. New, Simon Eaton, Keith R. F. Elliott, and Patti A. Quant
28. Developmental Comparison of Human and Rat Hepatic
Mitochondrial 3 Hydroxy 3 Methylglutaryl CoA Synthase 233
Carol V. Lascelles and Patti A. Quant
29. Regulation of the Ketogenic Enzyme Mitochondrial 3 Hydroxy
3 Methylglutaryl CoA Synthase in Astrocytes and Meningeal »
Fibroblasts: Implications in Normal Brain Development and
Seizure Neuropathologies 241
T. E. Cullingford, K. K. Bhakoo, S. Peuchen,
C. T. Dolphin, and J. B. Clark
30. Studies on Regulation of the Peroxisomal P Oxidation at the
3 Ketothiolase Step: Dissection of the Rat Liver Thiolase
B Gene Promoter 253
Norbert Latruffe, Valerie Nicolas Frances, Vijay Kumar Dasari,
and Takashi Osumi
31. Role and Organization of Peroxisomal p Oxidation 261
Paul P. Van Veldhoven and Guy P. Mannaerts
32. Hepatic oc Oxidation of Phytanic Acid: A Revised Pathway 273
Paul P. Van Veldhoven, Guy P. Mannaerts, Minne Casteels,
and Kathleen Croes
33. Functions and Dysfunctions of Peroxisomes in Fatty Acid
a and p Oxidation: New Insights 283
R. J. A. Wanders
t
34. Enzymology of P Oxidation of (Poly)unsaturated Fatty Acids 301
D. K. Novikov, K. T. Koivuranta, H. M. Helander,
S. A. Filppula, A. I. Yagi, Y. M. Qin, and K. J. Hiltunen
35. The Effect of P Oxidable and Non P Oxidable Thia Fatty
Acids on Fatty Acid Metabolism 311
Endre Dyroy, Lise Madsen, and Rolf K. Berge
36. EPA and DHA Possess Different Metabolic Properties 315
Lise Madsen, Endre Dyray, and Rolf Berge
37. The Use of [9,10 3H]Myristate, [9,10 3H]Palmitate and [9,10 3H]Oleate
for the Detection and Diagnosis of Medium and Long Chain
Fatty Acid Oxidation Disorders in Intact Cultured Fibroblasts 321
S. E. Olpin, N. J. Manning, R. J. Pollitt, J. R. Bonham,
M. Downing, and S. Clark
Contents xjj;
38. Rapid Diagnosis of Organic Acidemias and Fatty Acid Oxidation
Defects by Quantitative Electrospray Tandem MS
Acyl Carnitine Analysis in Plasma 327
P. Vreken, A. E. M. van Lint, A. H. Bootsma, H. Overmars,
R. J. A. Wanders, and A. H. van Gennip
39. Genetics of Carnitine Palmitoyltransferase II Deficiencies 339
Thomas Wieser, Marcus Deschauer, and Stephan Zierz
40. Identification of a Missense Mutation in a Patient with
Lethal Carnitine Acyl Carnitine Carrier Deficiency 347
L. Ulst, J. P. N. Ruiter, W. Oostheim, K. E. Niezen Koning,
F. Palmieri, and R. J. A. Wanders
41. MCAD Deficiency: Acylcarnitines (AC) by Tandem Mass
Spectrometry (MS MS) are Useful to Monitor Dietary Treatment . .. 353
J. E. Abdenur, N. A. Chamoles, N. Specola, A. B. Schenone,
L. Jorge, A. Guinle, C. I. Bernard, V. Levandowskiy, and S. Lavorgna
42. D Hydroxyacyl CoA Dehydrogenase Deficiency: Identification
of a New Peroxisomal Disorder with Implications for
Other Disorders of (3 Oxidation 365
E. G. van Grunsven, E. van Berkel, S. Denis, P. A. W. Mooijer,
and R. J. A. Wanders
43. Phytanoyl CoA Hydroxylase Deficiency: Enzymological and
Molecular Basis of Classical Refsum Disease 371
G. A. Jansen, S. Ferdinandusse, E. M. Hogenhout,
N. M. Verhoeven, C. Jakobs, and R. J. A. Wanders
44. Rationale for a Conditional Knockout Mouse Model to Study
Carnitine Palmitoyltransferase I Deficiencies 377
F. R. van der Leij, A. Drijfholt, and J. R. G. Kuipers
45. Biochemical Characterisation of Mutations of Human
Medium Chain Acyl CoA Dehydrogenase 387
Szabolcs Udvari, Peter Bross, Brage S. Andresen,
Niels Gregersen, and Paul C. Engel
46. Lessons Learned from the Mouse Model of Short Chain Acyl CoA
Dehydrogenase Deficiency 395
Philip A. Wood, C. Lisa Kelly Kurtz, Myron E. Hinsdale,
Doug A. Hamm, and William J. Rhead
Index 403
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| spelling | Current views of fatty acid oxidation and ketogenesis from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] ed. by Patti A. Quant ... New York [u.a.] Kluwer 1999 XVII, 414 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Advances in experimental medicine and biology 466 Fettsäurestoffwechsel (DE-588)4154234-4 gnd rswk-swf Biochemie (DE-588)4006777-4 gnd rswk-swf Ketonkörper (DE-588)4296663-2 gnd rswk-swf (DE-588)1071861417 Konferenzschrift 1998 London gnd-content Fettsäurestoffwechsel (DE-588)4154234-4 s DE-604 Ketonkörper (DE-588)4296663-2 s Biochemie (DE-588)4006777-4 s Quant, Patti A. Sonstige oth Advances in experimental medicine and biology 466 (DE-604)BV000003102 466 HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009120594&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Current views of fatty acid oxidation and ketogenesis from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] Advances in experimental medicine and biology Fettsäurestoffwechsel (DE-588)4154234-4 gnd Biochemie (DE-588)4006777-4 gnd Ketonkörper (DE-588)4296663-2 gnd |
| subject_GND | (DE-588)4154234-4 (DE-588)4006777-4 (DE-588)4296663-2 (DE-588)1071861417 |
| title | Current views of fatty acid oxidation and ketogenesis from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] |
| title_auth | Current views of fatty acid oxidation and ketogenesis from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] |
| title_exact_search | Current views of fatty acid oxidation and ketogenesis from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] |
| title_full | Current views of fatty acid oxidation and ketogenesis from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] ed. by Patti A. Quant ... |
| title_fullStr | Current views of fatty acid oxidation and ketogenesis from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] ed. by Patti A. Quant ... |
| title_full_unstemmed | Current views of fatty acid oxidation and ketogenesis from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] ed. by Patti A. Quant ... |
| title_short | Current views of fatty acid oxidation and ketogenesis |
| title_sort | current views of fatty acid oxidation and ketogenesis from organelles to point mutations proceedings of 4th fatty acid oxydation and ketogenesis conference held april 3 6 1998 in london england |
| title_sub | from organelles to point mutations ; [proceedings of 4th Fatty Acid Oxydation and Ketogenesis Conference, held April 3 - 6, 1998, in London, England] |
| topic | Fettsäurestoffwechsel (DE-588)4154234-4 gnd Biochemie (DE-588)4006777-4 gnd Ketonkörper (DE-588)4296663-2 gnd |
| topic_facet | Fettsäurestoffwechsel Biochemie Ketonkörper Konferenzschrift 1998 London |
| url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009120594&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
| volume_link | (DE-604)BV000003102 |
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