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Propionic acidemia in mice: Liver acyl-CoA levels and clinical course
期刊： MOLECULAR GENETICS AND METABOLISM, 2022; 135 (1)
Propionic acidemia (PA) is a severe autosomal recessive metabolic disease caused by deficiency of propionyl-CoA carboxylase (PCC). We studied PA trans......
Mitochondrial DNA maintenance defects: potential therapeutic strategies
期刊： MOLECULAR GENETICS AND METABOLISM, 2022; 137 (1-2)
Mitochondrial DNA (mtDNA) replication depends on the mitochondrial import of hundreds of nuclear encoded proteins that control the mitochondrial genom......
