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本文引用的文献
1
Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease.
Hum Mol Genet. 2012 Mar 1;21(5):1124-37. doi: 10.1093/hmg/ddr541. Epub 2011 Nov 17.
2
Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice.
Hum Mol Genet. 2012 Feb 1;21(3):526-35. doi: 10.1093/hmg/ddr482. Epub 2011 Oct 19.
3
ATGL-mediated fat catabolism regulates cardiac mitochondrial function via PPAR-α and PGC-1.
Nat Med. 2011 Aug 21;17(9):1076-85. doi: 10.1038/nm.2439.
4
A metabolic shift induced by a PPAR panagonist markedly reduces the effects of pathogenic mitochondrial tRNA mutations.
J Cell Mol Med. 2011 Nov;15(11):2317-25. doi: 10.1111/j.1582-4934.2010.01223.x.
5
Long-term follow-up of bezafibrate treatment in patients with the myopathic form of carnitine palmitoyltransferase 2 deficiency.
Clin Pharmacol Ther. 2010 Jul;88(1):101-8. doi: 10.1038/clpt.2010.55. Epub 2010 May 26.
6
Fat metabolism during exercise in patients with McArdle disease.
Neurology. 2009 Feb 24;72(8):718-24. doi: 10.1212/01.wnl.0000343002.74480.e4.
7
Bezafibrate for an inborn mitochondrial beta-oxidation defect.
N Engl J Med. 2009 Feb 19;360(8):838-40. doi: 10.1056/NEJMc0806334.
8
Potential of fibrates in the treatment of fatty acid oxidation disorders: revival of classical drugs?
J Inherit Metab Dis. 2006 Apr-Jun;29(2-3):341-2. doi: 10.1007/s10545-006-0266-3.
9
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 Sep 15;14(18):2695-703. doi: 10.1093/hmg/ddi303. Epub 2005 Aug 22.
10
Fuel utilization in subjects with carnitine palmitoyltransferase 2 gene mutations.
Ann Neurol. 2005 Jan;57(1):60-6. doi: 10.1002/ana.20320.
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