Bezafibrate upregulates carnitine palmitoyltransferase II expression and promotes mitochondrial energy crisis dissipation in fibroblasts of patients with influenza-associated encephalopathy

Molecular Genetics and Metabolism

Volumn 104, Issue 3, 2011, Pages 265-272

  Yao, Min ^a   Yao, Dengbin ^a   Yamaguchi, Miyoko ^a   Chida, Junji ^a   Yao, Dengfu ^b   Kido, Hiroshi ^a  

^a UNIVERSITY OF TOKUSHIMA   (Japan)

^b NANTONG UNIVERSITY   (China)

Author keywords

Bezafibrate; Carnitine palmitoyltransferase II; Energy crisis; Influenza associated encephalopathy; Mitochondrial membrane potential; Thermolabile variation

Indexed keywords

ADENOSINE TRIPHOSPHATE; BEZAFIBRATE; CARNITINE; CARNITINE PALMITOYLTRANSFERASE; CARNITINE PALMITOYLTRANSFERASE IA; CARNITINE PALMITOYLTRANSFERASE II; MESSENGER RNA; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR DELTA; UNCLASSIFIED DRUG;

ARTICLE; BRAIN DISEASE; CELL FUNCTION; CONTROLLED STUDY; DOWN REGULATION; ENZYME ACTIVITY; FATTY ACID OXIDATION; FIBROBLAST; GENE EXPRESSION REGULATION; HEAT STRESS; HUMAN; HUMAN CELL; INFLUENZA ASSOCIATED ENCEPHALOPATHY; MITOCHONDRIAL ENERGY TRANSFER; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRIAL RESPIRATION; NUCLEOTIDE SEQUENCE; PRIORITY JOURNAL; REAL TIME POLYMERASE CHAIN REACTION; TEMPERATURE DEPENDENCE; THERMAL INSTABILITY; THERMOSTABILITY; UPREGULATION;

ADENOSINE TRIPHOSPHATE; BASE SEQUENCE; BEZAFIBRATE; BLOTTING, WESTERN; BRAIN DISEASES, METABOLIC; CARNITINE; CARNITINE O-PALMITOYLTRANSFERASE; DNA PRIMERS; ENERGY METABOLISM; FIBROBLASTS; GENE EXPRESSION REGULATION; GENOMICS; HOT TEMPERATURE; HUMANS; INFLUENZA, HUMAN; JAPAN; MEMBRANE POTENTIAL, MITOCHONDRIAL; MICROSCOPY, FLUORESCENCE; MITOCHONDRIA; MOLECULAR SEQUENCE DATA; PEROXISOME PROLIFERATOR-ACTIVATED RECEPTORS; REAL-TIME POLYMERASE CHAIN REACTION; RNA, MESSENGER; SEQUENCE ANALYSIS, DNA; TIME FACTORS;

EID: 82455212976     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.07.009     Document Type: Article

References (29)

1. Morishima T., Togashi T., Yokota S., Okuno Y., Miyazaki C., Tashiro M., Okabe N. Encephalitis and encephalopathy associated with an influenza epidemic in Japan. Clin. Infect. Dis. 2002, 35:512-517.
2. Mizuguchi M., Abe J., Mikkaichi K., Noma S., Yoshida K., Yamanaka T., Kamoshita S. Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J. Neurol. Neurosurg. Psychiatry 1995, 58:555-561.
3. Mizuguchi M., Yamanouchi H., Ichiyama T., Shiomi M. Acute encephalopathy associated with influenza and other viral infections. Acta Neurol. Scand. 2007, 115:45-56.
4. Fujimoto S., Kobayashi M., Uemura O., Iwasa M., Ando T., Katoh T., Nakamura C., Maki N., Togari H., Wada Y. PCR on cerebrospinal fluid to show influenza-associated acute encephalopathy or encephalitis. Lancet 1998, 352:873-875.
5. Kasai T., Togashi T., Morishima T. Encephalopathy associated with influenza epidemics. Lancet 2000, 355:1558-1559.
6. Ichiyama T., Suenaga N., Kajimoto M., Tohyama J., Isumi H., Kubota M., Mori M., Furukawa S. Serum and CSF levels of cytokines in acute encephalopathy following prolonged febrile seizures. Brain Dev. 2008, 30:47-52.
7. Yokota S., Imagawa T., Miyamae T., Ito S., Nakajima S., Nezu A., Mori M. Hypothetical pathophysiology of acute encephalopathy and encephalitis related to influenza virus infection and hypothermia therapy. Pediatr. Int. 2000, 42:197-203.
8. McGarry J.D., Foster D.W. Systemic carnitine deficiency. N Engl J. Med. 1980, 303:1413-1415.
9. Bieber L.L. Carnitine. Annu. Rev. Biochem. 1988, 57:261-283.
10. Bonnefont J.P., Djouadi F., Prip-Buus C., Gobin S., Munnich A., Bastin J. Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects. Mol. Aspects Med. 2004, 25:495-520.
11. Chen Y., Mizuguchi H., Yao D., Ide M., Kuroda Y., Shigematsu Y., Yamaguchi S., Yamaguchi M., Kinoshita M., Kido H. Thermolabile phenotype of carnitine palmitoyltransferase II variations as a predisposing factor for influenza-associated encephalopathy. FEBS Lett. 2005, 579:2040-2044.
12. NCBI Single nucleotide polymorphism Available from URL:. http://www.ncbi.nlm.nih.gov/SNP/snpref.cgi?locusId=1376.
13. Yao D., Mizuguchi H., Yamaguchi M., Yamada H., Chida J., Shikata K., Kido H. Thermal instability of compound variants of carnitine palmitoyltransferase II and impaired mitochondrial fuel utilization in influenza-associated encephalopathy. Hum. Mutat. 2008, 29:718-727.
14. Dagher Z., Ruderman N., Tornheim K., Ido Y. Acute regulation of fatty acid oxidation and AMP-activated protein kinase in human umbilical vein endothelial cells. Circ. Res. 2001, 88:1276-1282.
15. Oldendorf W.H., Cornford M.E., Brown W.J. The large apparent work capability of the blood-brain barrier: a study of the mitochondrial content of capillary endothelial cells in brain and other tissues of the rat. Ann. Neurol. 1977, 1:409-417.
16. Wang S., Le T.Q., Kurihara N., Chida J., Cisse Y., Yano M., Kido H. Influenza virus-cytokine-protease cycle in the pathogenesis of vascular hyperpermeability in severe influenza. J. Infect. Dis. 2010, 202:991-1001.
17. Pan H., Yamada H., Chida J., Wang S., Yano M., Yao M., Zhu J., Kido H. Up-regulation of ectopic trypsins in the myocardium by influenza A virus infection triggers acute myocarditis. Cardiovasc. Res. 2011, 89:595-603.
18. Kamei S., Sekizawa T., Shiota H., Mizutani T., Itoyama Y., Takasu T., Morishima T., Hirayanagi K. Evaluation of combination therapy using aciclovir and corticosteroid in adult patients with herpes simplex virus encephalitis. J. Neurol. Neurosurg. Psychiatry 2005, 76:1544-1549.
19. Berger J., Moller D.E. The mechanisms of action of PPARs. Annu. Rev. Med. 2002, 53:409-435.
20. Lee C.H., Olson P., Evans R.M. Minireview: lipid metabolism, metabolic diseases, and peroxisome proliferator-activated receptors. Endocrinology 2003, 144:2201-2207.
21. Vamecq J., Latruffe N. Medical significance of peroxisome proliferator-activated receptors. Lancet 1999, 354:141-148.
22. Djouadi F., Aubey F., Schlemmer D., Bastin J. Peroxisome proliferator activated receptors δ (PPARδ) agonist but not PPARα corrects carnitine palmitoyl transferase 2 deficiency in human muscle cells. J. Clin. Endocrinol. Metab. 2005, 90:1791-1797.
23. Bonnefont J.P., Bastin J., Laforêt P., Aubey F., Mogenet A., Romano S., Ricquier D., Gobin-Limballe S., Vassault A., Behin A., Eymard B., Bresson J.L., Djouadi F. Long-term follow-up of bezafibrate treatment in patients with the myopathic form of carnitine palmitoyltransferase 2 deficiency. Clin. Pharmacol. Ther. 2010, 88:101-108.
24. Laemmli U.K. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature 1970, 227:680-685.
25. Saudubray J.M., Coude F.X., Demaugre F., Johnson C., Gibson K.M., Nyham W.L. Oxidation of fatty acids in cultured fibroblasts: a model system for the detection and study of defects in oxidation. Pediatr. Res. 1982, 16:877-881.
26. Olpin S.E., Allen J., Bonham J.R., Clark S., Clayton P.T., Calvin J., Downing M., Ives K., Jones S., Manning N.J., Pollitt R.J., Standing S.J., Tanner M.S. Features of carnitine palmitoyltransferase type I deficiency. J. Inherit. Metab. Dis. 2001, 24:35-42.
27. Lee S., Jeong S.Y., Lim W.C., Kim S., Park Y.Y., Sun X., Youle R.J., Cho H. Mitochondrial fission and fusion mediators, hFis1 and OPA1, modulate cellular senescence. J. Biol. Chem. 2007, 282:22977-22983.
28. Shinohara M., Saitoh M., Takanashi J., Yamanouchi H., Kubota M., Goto T., Kikuchi M., Shiihara T., Yamanaka G., Mizuguchi M. Carnitine palmitoyl transferase II polymorphism is associated with multiple syndromes of acute encephalopathy with various infectious diseases. Brain Dev. 2010, PMID: 20934285.
29. Kubota M., Chida J., Hoshino H., Ozawa H., Koide A., Kashii H., Koyama A., Mizuno Y., Hoshino A., Yamaguchi M., Yao D., Yao M., Kido H. Thermolabile CPT II variants and low blood ATP levels are closely related to severity of acute encephalopathy in Japanese children. Brain Dev. 2011, PMID: 21277129.