Reduced mitochondrial DNA and OXPHOS protein content in skeletal muscle of children with cerebral palsy

Aim To provide a detailed gene and protein expression analysis related to mitochondrial biogenesis assess content in skeletal muscle of children with cerebral palsy (CP). Method Biceps brachii samples were collected from 19 CP (mean [SD] age 15y 4mo [2y 6mo], range 9–18y, 16 males, three females) 10 typically developing comparison [4y], 7–21y, eight two females). Gene (quantitative reverse transcription polymerase chain reaction [PCR]), DNA (mtDNA) genomic ratio PCR), abundance (western blotting) analyzed. Microarray data sets (CP/aging/bed rest) analyzed focused query investigating metabolism- mitochondria-related networks. Results The mtDNA was lower the compared group (−23%, p=0.002). Out five investigated complexes respiratory chain, we observed levels all (I, III, IV, V, −20% −37%; p<0.05) except complex II. Total peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC1α) messenger RNA (p<0.004), isoforms PGC1α1 (p=0.05), PGC1α4 (p<0.001) reduced CP. Transcriptional similarities between CP, aging, 90 days’ bed rest. Interpretation Mitochondrial biogenesis, mtDNA, oxidative phosphorylation are muscle. pathways shared aging long-term unloading suggests metabolic dysregulation which may guide therapeutic strategies for combatting pathology. What this paper adds Cerebral (CP) contains fewer energy-generating organelles than is similar