Reversal of Defective Mitochondrial Biogenesis in Limb-Girdle Muscular Dystrophy 2D by Independent Modulation of Histone and PGC-1α Acetylation

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• 作者：Sarah Pambianco¹ ; ⁹ ; Matteo Giovarelli¹ ; ⁹ ; Cristiana Perrotta¹ ; Silvia Zecchini² ; Davide Cervia¹ ; ³ ; Ilaria Di Renzo¹ ; Claudia Moscheni¹ ; Michela Ripolone⁴ ; Raffaella Violano⁴ ; Maurizio Moggio⁴ ; Maria Teresa Bassi⁵ ; Pier Lorenzo Puri⁶ ; ⁷ ; Lucia Latella⁶ ; ⁸ ; Emilio Clementi² ; ⁵ ; ^{emilio.clementi@unimi.it} ; Clara De Palma² ; ¹⁰ ; ^{clara.depalma@unimi.it}
• 关键词：muscular dystrophy ; mitochondrial biogenesis ; histone acetylation ; fatty acid oxidation
• 刊名：Cell Reports
• 出版年：2016
• 出版时间：13 December 2016
• 年：2016
• 卷：17
• 期：11
• 页码：3010-3023
• 全文大小：4218 K
• 卷排序：17

文摘

LGMD-2D patients and α-SG null mice display reduced mitochondrial content Reduced acetylation of the PGC-1α promoter causes the mitochondrial defect Trichostatin A reverses chromatin modifications, restoring mitochondrial biogenesis Nitric oxide activates a salvage pathway that shunts faulty mitochondrial biogenesis