Fatty acid accumulation and resulting PPARα activation in fibroblasts due to trifunctional protein deficiency

PPAR Research

Volumn , Issue , 2012, Pages

  Wakabayashi, Masato ^a,b   Kamijo, Yuji ^a,c   Nakajima, Takero ^a   Tanaka, Naoki ^a   Sugiyama, Eiko ^a,d   Yangyang, Tian ^a   Kimura, Takefumi ^a,c   Aoyama, Toshifumi ^a  

^a SHINSHU UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^b NAGANO RED CROSS HOSPITAL   (Japan)

^c SHINSHU UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^d Nagano Prefectural College   (Japan)

Author keywords

[No Author keywords available]

Indexed keywords

3 [3 TERT BUTYLTHIO 1 (4 CHLOROBENZYL) 5 ISOPROPYL 2 INDOLYL] 2,2 DIMETHYLPROPIONIC ACID; ACYL COENZYME A DEHYDROGENASE; FATTY ACID; FENOFIBRATE; PALMITOYL COENZYME A; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR ALPHA;

ARTICLE; CATALYSIS; CELL PROLIFERATION; CLINICAL ARTICLE; CLINICAL FEATURE; CONTROLLED STUDY; DNA BINDING; ENZYME ACTIVATION; ENZYME ACTIVITY; FATTY ACID METABOLISM; FATTY ACID OXIDATION; HEPATOMEGALY; HUMAN; HUMAN CELL; HYPERTROPHIC CARDIOMYOPATHY; MALE; MITOCHONDRION; MYOPATHY; OXIDATIVE STRESS; PROTEIN BINDING; PROTEIN DEFICIENCY; PROTEIN EXPRESSION; SKIN FIBROBLAST; TRIFUNCTIONAL PROTEIN DEFICIENCY;

EID: 84862293756     PISSN: 16874757     EISSN: 16874765     Source Type: Journal    
DOI: 10.1155/2012/371691     Document Type: Article

References (32)

1. Kamijo Y., Hora K., Kono K., Takahashi K., Higuchi M., Ehara T., Kiyosawa K., Shigematsu H., Gonzalez F. J., Aoyama T., PPAR protects proximal tubular cells from acute fatty acid toxicity Journal of the American Society of Nephrology 2007 18 12 3089 3100 (Pubitemid 350223969)
2. Takahashi K., Kamijo Y., Hora K., Hashimoto K., Higuchi M., Nakajima T., Ehara T., Shigematsu H., Gonzalez F. J., Aoyama T., Pretreatment by low-dose fibrates protects against acute free fatty acid-induced renal tubule toxicity by counteracting PPAR deterioration Toxicology and Applied Pharmacology 2011 252 3 237 249
3. Tanaka N., Moriya K., Kiyosawa K., Koike K., Gonzalez F. J., Aoyama T., PPAR activation is essential for HCV core protein-induced hepatic steatosis and hepatocellular carcinoma in mice Journal of Clinical Investigation 2008 118 2 683 694 (Pubitemid 351206557)
4. Tanaka N., Moriya K., Kiyosawa K., Koike K., Aoyama T., Hepatitis C virus core protein induces spontaneous and persistent activation of peroxisome proliferator-activated receptor in transgenic mice: implications for HCV-associated hepatocarcinogenesis International Journal of Cancer 2008 122 1 124 131 (Pubitemid 350209986)
5. Orii K. E., Aoyama T., Wakui K., Fukushima Y., Miyajima H., Yamaguchi S., Orii T., Kondo N., Hashimoto T., Genomic and mutational analysis of the mitochondrial trifunctional protein -subunit (HADHB) gene in patients with trifunctional protein deficiency Human Molecular Genetics 1997 6 8 1215 1224 (Pubitemid 27351060)
6. Jackson S., Kler R. S., Bartlett K., Briggs H., Bindoff L. A., Pourfarzam M., Gardner-Medwin D., Turnbull D. M., Combined enzyme defect of mitochondrial fatty acid oxidation Journal of Clinical Investigation 1992 90 4 1219 1225
7. Wanders R. J. A., Ijlst L. I, Poggi F., Bonnefont J. P., Munnich A., Brivet M., Rabier D., Saudubray J. M., Human trifunctional protein deficiency: a new disorder of mitochondrial fatty acid -oxidation Biochemical and Biophysical Research Communications 1992 188 3 1139 1145
8. Kehrer J. P., Biswal S. S., La E., Thuillier P., Datta K., Fischer S. M., Vanden Heuvel J. P., Inhibition of peroxisome-proliferator-activated receptor (PPAR) by MK886 Biochemical Journal 2001 356 3 899 906 (Pubitemid 32588464)
9. Ushikubo S., Aoyama T., Kamijo T., Wanders R. J. A., Rinaldo P., Vockley J., Hashimoto T., Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both -and -subunits American Journal of Human Genetics 1996 58 5 979 988 (Pubitemid 26115169)
10. Ijlst L. I, Wanders R. J. A., Ushikubo S., Kamijo T., Hashimoto T., Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the -subunit of the mitochondrial trifunctional protein Biochimica Et Biophysica Acta 1994 1215 3 347 350
11. Furuta S., Mayazawa S., Hashimoto T., Purification and properties of rat liver acyl-CoA dehydrogenases and electron transfer flavoprotein Journal of Biochemistry 1981 90 6 1739 1750
12. Lehman T. C., Hale D. E., Bhala A., Thorpe C., An acyl-coenzyme A dehydrogenase assay utilizing the ferricenium ion Analytical Biochemistry 1990 186 2 280 284 (Pubitemid 20153505)
13. Aoyama T., Yamano S., Waxman D. J., Lapenson D. P., Meyer U. A., Fischer V., Tyndale R., Inaba T., Kalow W., Gelboin H. V., Gonzalez F. J., Cytochrome P-450 hPCN3, a novel cytochrome P-450 IIIA gene product that is differentially expressed in adult human liver. cDNA and deduced amino acid sequence and distinct specificities of cDNA-expressed hpcn1 and hPCN3 for the metabolism of steroid hormones and cyclosporine Journal of Biological Chemistry 1989 264 18 10388 10395 (Pubitemid 19161599)
14. Aoyama T., Ueno I., Kamijo T., Hashimoto T., Rat very-long-chain acyl-CoA dehydrogenase, a novel mitochondrial acyl-CoA dehydrogenase gene product, is a rate-limiting enzyme in long-chain fatty acid -oxidation system. cDNA and deduced amino acid sequence and distinct specificities of the cDNA-expressed protein Journal of Biological Chemistry 1994 269 29 19088 19094
15. Aoyama T., Peters J. M., Iritani N., Nakajima T., Furihata K., Hashimoto T., Gonzalez F. J., Altered constitutive expression of fatty acid-metabolizing enzymes in mice lacking the peroxisome proliferator-activated receptor (PPAR ) Journal of Biological Chemistry 1998 273 10 5678 5684 (Pubitemid 28124039)
16. Kamijo Y., Hora K., Tanaka N., Usuda N., Kiyosawa K., Nakajima T., Gonzalez F. J., Aoyama T., Identification of functions of peroxisome proliferator-activated receptor in proximal tubules Journal of the American Society of Nephrology 2002 13 7 1691 1702 (Pubitemid 34700593)
17. Desvergne B., Wahli W., Peroxisome proliferator-activated receptors: nuclear control of metabolism Endocrine Reviews 1999 20 5 649 688 (Pubitemid 30650602)
18. Duplus E., Glorian M., Forest C., Fatty acid regulation of gene transcription Journal of Biological Chemistry 2000 275 40 30749 30752
19. Gervois P., Torra I. P., Fruchart J. C., Staels B., Regulation of lipid and lipoprotein metabolism by PPAR activators Clinical Chemistry and Laboratory Medicine 2000 38 1 3 11 (Pubitemid 30162787)
20. Hara A., Radin N. S., Lipid extraction of tissues with a low-toxicity solvent Analytical Biochemistry 1978 90 1 420 426 (Pubitemid 9062124)
21. Kliewer S. A., Sundseth S. S., Jones S. A., Brown P. J., Wisely G. B., Koble C. S., Devchand P., Wahli W., Willson T. M., Lenhard J. M., Lehmann J. M., Fatty acids and eicosanoids regulate gene expression through direct interactions with peroxisome proliferator-activated receptors and Proceedings of the National Academy of Sciences of the United States of America 1997 94 9 4318 4323 (Pubitemid 27194239)
22. Forman B. M., Chen J., Evans R. M., Hypolipidemic drugs, polyunsaturated fatty acids, and eicosanoids are ligands for peroxisome proliferator-activated receptors and Proceedings of the National Academy of Sciences of the United States of America 1997 94 9 4312 4317 (Pubitemid 27194238)
23. Xu H. E., Lambert M. H., Montana V. G., Parks D. J., Blanchard S. G., Brown P. J., Sternbach D. D., Lehmann J. M., Wisely G. B., Willson T. M., Kliewer S. A., Milburn M. V., Molecular recognition of fatty acids by peroxisome proliferator-activated receptors Molecular Cell 1999 3 3 397 403 (Pubitemid 29290680)
24. Rakhshandehroo M., Knoch B., Muller M., Kersten S., Peroxisome proliferator-activated receptor alpha target genes PPAR Research 2010 2010
25. Braissant O., Foufelle F., Scotto C., Dauca M., Wahli W., Differential expression of peroxisome proliferator-activated receptors (PPARs): tissue distribution of PPAR-,-, and- in the adult rat Endocrinology 1996 137 1 354 366 (Pubitemid 26005011)
26. Coates P. M., Hale D. E., Finocchiaro G., Tanaka K., Winter S. C., Genetic deficiency of short-chain acyl-coenzyme A dehydrogenase in cultured fibroblasts from a patient with muscle carnitine deficiency and severe skeletal muscle weakness Journal of Clinical Investigation 1988 81 1 171 175 (Pubitemid 18092249)
27. Naito E., Indo Y., Tanaka K., Short chain acyl-coenzyme A dehydrogenase (SCAD) deficiency. immunochemical demonstration of molecular heterogeneity due to variant SCAD with differing stability Journal of Clinical Investigation 1989 84 5 1671 1674 (Pubitemid 19272258)
28. Rinaldo P., O'Shea J. J., Coates P. M., Hale D. E., Stanley C. A., Tanaka K., Medium-chain acyl-CoA dehydrogenase deficiency: diagnosis by stable-isotope dilution measurement of urinary n-hexanoylglycine and 3-phenylpropionylglycine New England Journal of Medicine 1988 319 20 1308 1313 (Pubitemid 19003392)
29. Coates P. M., Indo Y., Young D., Hale D. E., Tanaka K., Immunochemical characterization of variant medium-chain acyl-CoA dehydrogenase in fibroblasts from patients with medium-chain acyl-CoA dehydrogenase deficiency Pediatric Research 1992 31 1 34 38
30. Aoyama T., Uchida Y., Kelley R. I., Marble M., Hofman K., Tonsgard J. H., Rhead W. J., Hashimoto T., A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase Biochemical and Biophysical Research Communications 1993 191 3 1369 1372 (Pubitemid 23231641)
31. Aoyama T., Souri M., Ushikubo S., Kamijo T., Yamaguchi S., Kelley R. I., Rhead W. J., Uetake K., Tanaka K., Hashimoto T., Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients Journal of Clinical Investigation 1995 95 6 2465 2473
32. Aoyama T., Souri M., Ueno I., Kamijo T., Yamaguchi S., Rhead W. J., Tanaka K., Hashimoto T., Cloning of human very-long-chain acyl-coenzyme A dehydrogenase and molecular characterization of its deficiency in two patients American Journal of Human Genetics 1995 57 2 273 283