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Volumn 107, Issue 1-2, 2012, Pages 87-91

Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: Evaluation by in vitro probe acylcarnitine assay

Author keywords

Bezafibrate; Hypolipidemic drug; In vitro probe acylcarnitine assay; Mitochondrial fatty acid oxidation disorder; New treatment; Peroxisome proliferation activator receptor

Indexed keywords

ACYLCARNITINE; BEZAFIBRATE; CARNITINE PALMITOYLTRANSFERASE; FATTY ACID; LONG CHAIN ACYL COENZYME A DEHYDROGENASE; MEDIUM CHAIN ACYL COENZYME A DEHYDROGENASE;

EID: 84866151057     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2012.07.004     Document Type: Article
Times cited : (49)

References (22)
  • 1
    • 39649108670 scopus 로고    scopus 로고
    • Expanded newborn screening: implications for genomic medicine
    • McCabe L.L., McCabe E.R.B. Expanded newborn screening: implications for genomic medicine. Annu. Rev. Med. 2008, 59:163-175.
    • (2008) Annu. Rev. Med. , vol.59 , pp. 163-175
    • McCabe, L.L.1    McCabe, E.R.B.2
  • 4
    • 49149109956 scopus 로고    scopus 로고
    • Mitochondrial fatty-acid oxidation disorders
    • Kompare M., Rizzo W.B. Mitochondrial fatty-acid oxidation disorders. Semin. Pediatr. Neurol. 2008, 15:140-149.
    • (2008) Semin. Pediatr. Neurol. , vol.15 , pp. 140-149
    • Kompare, M.1    Rizzo, W.B.2
  • 5
    • 77957554125 scopus 로고    scopus 로고
    • Pathophysiology of fatty acid oxidation disorders
    • Bennett M.J. Pathophysiology of fatty acid oxidation disorders. J. Inherit. Metab. Dis. 2010, 33:533-537.
    • (2010) J. Inherit. Metab. Dis. , vol.33 , pp. 533-537
    • Bennett, M.J.1
  • 7
    • 0037057759 scopus 로고    scopus 로고
    • A method for quantitative acylcarnitine profiling in human skin fibroblasts using unlabelled palmitic acid: diagnosis of fatty acid oxidation disorders and differentiation between biochemical phenotypes of MCAD deficiency
    • Okun J.G., Kolker S., Schulze A., Kohlmuller D., Olgemoller K., Linder M., Hoffmann G.F., Wanders R.J.A., Mayatepek E. A method for quantitative acylcarnitine profiling in human skin fibroblasts using unlabelled palmitic acid: diagnosis of fatty acid oxidation disorders and differentiation between biochemical phenotypes of MCAD deficiency. Biochim. Biophys. Acta 2002, 1584:91-98.
    • (2002) Biochim. Biophys. Acta , vol.1584 , pp. 91-98
    • Okun, J.G.1    Kolker, S.2    Schulze, A.3    Kohlmuller, D.4    Olgemoller, K.5    Linder, M.6    Hoffmann, G.F.7    Wanders, R.J.A.8    Mayatepek, E.9
  • 8
    • 0036389795 scopus 로고    scopus 로고
    • Quantitative fibroblast acylcarnitine profiles in mitochondrial fatty acid beta-oxidation defects: phenotype/metabolite correlations
    • Sim K.G., Carpenter K., Hammond J., Christodoulou J., Wilcken B. Quantitative fibroblast acylcarnitine profiles in mitochondrial fatty acid beta-oxidation defects: phenotype/metabolite correlations. Mol. Genet. Metab. 2002, 76:327-334.
    • (2002) Mol. Genet. Metab. , vol.76 , pp. 327-334
    • Sim, K.G.1    Carpenter, K.2    Hammond, J.3    Christodoulou, J.4    Wilcken, B.5
  • 9
    • 0032489437 scopus 로고    scopus 로고
    • Altered constitutive expression of fatty acid-metabolizing enzymes in mice lacking the peroxisome proliferator-activated receptor alpha (PPARalpha)
    • 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 alpha (PPARalpha). J. Biol. Chem. 1998, 273:5678-5684.
    • (1998) J. Biol. Chem. , vol.273 , pp. 5678-5684
    • Aoyama, T.1    Peters, J.M.2    Iritani, N.3    Nakajima, T.4    Furihata, K.5    Hashimoto, T.6    Gonzalez, F.J.7
  • 10
    • 0141788824 scopus 로고    scopus 로고
    • Correction of fatty acid oxidation I carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate
    • Djouadi F., Bonnefont J.P., Thuillier L., Droin V., Khadom N., Munnich A., Bastin J. Correction of fatty acid oxidation I carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate. Pediatr. Res. 2003, 54:446-451.
    • (2003) Pediatr. Res. , vol.54 , pp. 446-451
    • Djouadi, F.1    Bonnefont, J.P.2    Thuillier, L.3    Droin, V.4    Khadom, N.5    Munnich, A.6    Bastin, J.7
  • 13
    • 77951022487 scopus 로고    scopus 로고
    • Effect of heat stress and bezafibrate on mitochondrial β-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay
    • Li H., Fukuda S., Hasegawa Y., Kobayashi H., Purevsuren J., Mushimoto Y., Yamaguchi S. Effect of heat stress and bezafibrate on mitochondrial β-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay. Brain Dev. 2010, 32:362-370.
    • (2010) Brain Dev. , vol.32 , pp. 362-370
    • Li, H.1    Fukuda, S.2    Hasegawa, Y.3    Kobayashi, H.4    Purevsuren, J.5    Mushimoto, Y.6    Yamaguchi, S.7
  • 17
    • 77957748796 scopus 로고    scopus 로고
    • Disorders of muscle lipid metabolism: diagnostic and therapeutic challenges
    • Laforêt P., Vianey-Saban C. Disorders of muscle lipid metabolism: diagnostic and therapeutic challenges. Neuromuscul. Disord. 2010, 20:693-700.
    • (2010) Neuromuscul. Disord. , vol.20 , pp. 693-700
    • Laforêt, P.1    Vianey-Saban, C.2
  • 18
    • 0036171573 scopus 로고    scopus 로고
    • Defects of mitochondrial beta-oxidation: a growing group of disorders
    • Vockley J., Whiteman D.A. Defects of mitochondrial beta-oxidation: a growing group of disorders. Neuromuscul. Disord. 2002, 12:235-246.
    • (2002) Neuromuscul. Disord. , vol.12 , pp. 235-246
    • Vockley, J.1    Whiteman, D.A.2
  • 19
    • 24944563136 scopus 로고    scopus 로고
    • Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders
    • Djouadi F., Aubey F., Schlemmer D., Ruiter J.P., Wanders R.J., Strauss A.W., Bastin J. Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders. Hum. Mol. Genet. 2005, 14:2695-2703.
    • (2005) Hum. Mol. Genet. , vol.14 , pp. 2695-2703
    • Djouadi, F.1    Aubey, F.2    Schlemmer, D.3    Ruiter, J.P.4    Wanders, R.J.5    Strauss, A.W.6    Bastin, J.7
  • 21
    • 60849099038 scopus 로고    scopus 로고
    • Bezafibrate for treatment of an inborn mitochondrial b-oxidation defect
    • Bonnefont J.P., Bastin J., Behin A., Djouadi F. Bezafibrate for treatment of an inborn mitochondrial b-oxidation defect. N. Engl. J. Med. 2009, 360:838-840.
    • (2009) N. Engl. J. Med. , vol.360 , pp. 838-840
    • Bonnefont, J.P.1    Bastin, J.2    Behin, A.3    Djouadi, F.4
  • 22
    • 79955442484 scopus 로고    scopus 로고
    • Exposure to resveratrol triggers pharmacological correction of fatty acid utilization in human fatty acid oxidation-deficient fibroblasts
    • Bastin J., Lopes-Costa A., Djouadi F. Exposure to resveratrol triggers pharmacological correction of fatty acid utilization in human fatty acid oxidation-deficient fibroblasts. Hum. Mol. Genet. 2011, 20:2048-2057.
    • (2011) Hum. Mol. Genet. , vol.20 , pp. 2048-2057
    • Bastin, J.1    Lopes-Costa, A.2    Djouadi, F.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.