刊名:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
出版年:2017
出版时间:January 2017
年:2017
卷:1863
期:1
页码:284-291
全文大小:710 K
卷排序:1863
文摘
MELAS syndrome is associated with mitochondrial complex I deficiency and disassembly. Ketone bodies improved complex I assembly and activity in MELAS neuronal cells. Ketone bodies increase ATP synthesis and mtDNA copy number in treated mutant cells. Ketone bodies may represent an alternative treatment for MELAS syndrome.