ALS-causing mutations differentially affect PGC-1α expression and function in the brain vs. peripheral tissues

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• 作者：Hanna Bayer^a ; ^{hanna.bayer@uni-ulm.de} ; Kerstin Lang^a ; ^{Kerstin.lang@uni-ulm.de} ; Eva Buck^a ; ^{eva.buck@uni-ulm.de} ; Julia Higelin^b ; ^{Julia.higelin@uni-ulm.de} ; Lara Barteczko^a ; ^{Lara.barteczko@uni-ulm.de} ; Noemi Pasquarelli^a ; ^{Noemi.pasquarelli@uni-ulm.de} ; Jasmin Sprissler^a ; ^{Jasmin.sprissler@uni-ulm.de} ; Tanja Lucas^c ; ^{Tanjalucas@web.de} ; Karlheinz Holzmann^d ; ^{karlheinz.holzmann@uni-ulm.de} ; Maria Demestre^b ; ^{maria.demestre@uni-ulm.de} ; Katrin S. Lindenberg^a ; ^{katrin.lindenberg@uni-ulm.de} ; Karin M. Danzer^a ; ^{karin.danzer@uni-ulm.de} ; Tobias Boeckers^b ; ^{tobias.boeckers@uni-ulm.de} ; Albert C. Ludolph^a ; ^{albert.ludolph@uni-ulm.de} ; Luc Dupuis^e ; ^f ; ^{ldupuis@neuro-cnrs.unistra.fr} ; Patrick Weydt^a ; ^g ; ^{patrick.weydt@uni-bonn.de} ; Anke Witting^a ; ^{anke.witting@uni-ulm.de}
• 关键词：ALS ; amyotrophic lateral sclerosis ; PGC-1α ; peroxisome-proliferator activated receptor gamma coactivator)-1α ; SOD1 ; superoxide dismutase 1 ; SOD3 ; superoxide dismutase 3 ; FUS/TLS ; fused in sarcoma/translocated in sarcoma
• 刊名：Neurobiology of Disease
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：97
• 期：part_PA
• 页码：36-45
• 全文大小：1333 K
• 卷排序：97

文摘

Metabolic dysregulation in ALS is unraveled on a molecular level in vitro and in vivo (85). Lactate triggers neuroprotective signals in the brain (54). ALS-mutations impair the PGC-1α response in patient-derived iPS cells (70). Metabolic responses differ fundamentally in peripheral vs. central ALS-tissues (79).