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Reduced mitochondrial DNA and OXPHOS protein content in skeletal muscle of children with cerebral palsy
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2021 (English)In: Developmental Medicine & Child Neurology, ISSN 0012-1622, E-ISSN 1469-8749, Vol. 63, no 10, p. 1204-1212Article in journal (Refereed) Published
Abstract [en]

AIM: To provide a detailed gene and protein expression analysis related to mitochondrial biogenesis and assess mitochondrial content in skeletal muscle of children with cerebral palsy (CP).

METHOD: Biceps brachii muscle samples were collected from 19 children with CP (mean [SD] age 15y 4mo [2y 6mo], range 9-18y, 16 males, three females) and 10 typically developing comparison children (mean [SD] age 15y [4y], range 7-21y, eight males, two females). Gene expression (quantitative reverse transcription polymerase chain reaction [PCR]), mitochondrial DNA (mtDNA) to genomic DNA ratio (quantitative PCR), and protein abundance (western blotting) were analyzed. Microarray data sets (CP/aging/bed rest) were analyzed with a focused query investigating metabolism- and mitochondria-related gene networks.

RESULTS: The mtDNA to genomic DNA ratio was lower in the children with CP compared to the typically developing group (-23%, p=0.002). Out of five investigated complexes in the mitochondrial respiratory chain, we observed lower protein levels of all complexes (I, III, IV, V, -20% to -37%; p<0.05) except complex II. Total peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC1 alpha) messenger RNA (p<0.004), isoforms PGC1 alpha 1 (p=0.05), and PGC1 alpha 4 (p<0.001) were reduced in CP. Transcriptional similarities were observed between CP, aging, and 90 days' bed rest.

INTERPRETATION: Mitochondrial biogenesis, mtDNA, and oxidative phosphorylation protein content are reduced in CP muscle compared with typically developing muscle. Transcriptional pathways shared between aging and long-term unloading suggests metabolic dysregulation in CP, which may guide therapeutic strategies for combatting CP muscle pathology.

Place, publisher, year, edition, pages
John Wiley & Sons, 2021. Vol. 63, no 10, p. 1204-1212
National Category
Neurology Pediatrics Physiology
Identifiers
URN: urn:nbn:se:umu:diva-187302DOI: 10.1111/dmcn.14964ISI: 000666868200001PubMedID: 34176131Scopus ID: 2-s2.0-85114218830OAI: oai:DiVA.org:umu-187302DiVA, id: diva2:1592216
Available from: 2021-09-08 Created: 2021-09-08 Last updated: 2021-09-14Bibliographically approved

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Stål, Per

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von Walden, FerdinandEnglund, DavisFernandez-Gonzalo, RodrigoPingel, JessicaStål, PerPontén, Eva
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