A metabolic shift induced by a PPAR panagonist markedly reduces the effects of pathogenic mitochondrial tRNA mutations

PGC-1α, SIRT1 and AMPK, an energy sensing network that controls energy expenditure

(2009) Carles Cantó et al.   CURRENT OPINION IN LIPIDOLOGY

PGC-1α/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders

(2009) Sarika Srivastava et al.   HUMAN MOLECULAR GENETICS

Endurance exercise is protective for mice with mitochondrial myopathy

(2009) Tina Wenz et al.   JOURNAL OF APPLIED PHYSIOLOGY

Mitochondrial Disorders in the Nervous System

(2008) Salvatore DiMauro et al.   Annual Review of Neuroscience

PGC-1α-mediated regulation of gene expression and metabolism: implications for nutrition and exercise prescriptions

(2008) Carley R. Benton et al.   Applied Physiology Nutrition and Metabolism

RETRACTED: Activation of the PPAR/PGC-1α Pathway Prevents a Bioenergetic Deficit and Effectively Improves a Mitochondrial Myopathy Phenotype

(2008) Tina Wenz et al.   Cell Metabolism

Protein Synthesis and Assembly in Mitochondrial Disorders

(2008) Xochitl Perez-Martinez et al.   CURRENT TOPICS IN MEDICINAL CHEMISTRY

The A3243G tRNALeu(UUR) MELAS mutation causes amino acid misincorporation and a combined respiratory chain assembly defect partially suppressed by overexpression of EFTu and EFG2

(2008) F. Sasarman et al.   HUMAN MOLECULAR GENETICS

Activation of Peroxisome Proliferator-Activated Receptor Pathway Stimulates the Mitochondrial Respiratory Chain and Can Correct Deficiencies in Patients’ Cells Lacking Its Components

(2008) Jean Bastin et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Overexpression of human mitochondrial valyl tRNA synthetase can partially restore levels of cognate mt-tRNAVal carrying the pathogenic C25U mutation

(2008) Joanna Rorbach et al.   NUCLEIC ACIDS RESEARCH

Transcriptional Paradigms in Mammalian Mitochondrial Biogenesis and Function

(2008) Richard C. Scarpulla   PHYSIOLOGICAL REVIEWS