ArticleAntioxidants & redox signaling2011
Prion protein expression and functional importance in skeletal muscle.
Article in Antioxidants & redox signaling, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 16 citations in OpenAlex.
- Prnp Deletion Mitigates Muscle Fiber Type-Specific Sarcopenia Induced by Prion Infection in Mice.Immunity, inflammation and disease · 2026Article
- PrPJournal of cachexia, sarcopenia and muscle · 2025Article
- Targeted brain-specific tauopathy compromises peripheral skeletal muscle integrity and function.Aging brain · 2024Article
- Excess PrPNature communications · 2023Article
- Targeted brain-specific tauopathy compromises peripheral skeletal muscle integrity and function.bioRxiv : the preprint server for biology · 2023Article
- Cellular prion protein dysfunction in a prototypical inherited metabolic myopathy.Cellular and molecular life sciences : CMLS · 2021Article
- Neutral sphingomyelinase-3 mediates TNF-stimulated oxidant activity in skeletal muscle.Redox biology · 2014Article
- Up-regulation of mRNA ventricular PRNP prion protein gene expression in air pollution highly exposed young urbanites: endoplasmic reticulum stress, glucose regulated protein 78, and nanosized particles.International journal of molecular sciences · 2013Article
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
unlabelledSkeletal muscle expresses prion protein (PrP) that buffers oxidant activity in neurons.
aimsWe hypothesize that PrP deficiency would increase oxidant activity in skeletal muscle and alter redox-sensitive functions, including contraction and glucose uptake. We used real-time polymerase chain reaction and Western blot analysis to measure PrP mRNA and protein in human diaphragm, five murine muscles, and muscle-derived C2C12 cells. Effects of PrP deficiency were tested by comparing PrP-deficient mice versus wild-type mice and morpholino-knockdown versus vehicle-treated myotubes. Oxidant activity (dichlorofluorescin oxidation) and specific force were measured in murine diaphragm fiber bundles.
resultsPrP content differs among mouse muscles (gastrocnemius>extensor digitorum longus, EDL>tibialis anterior, TA; soleus>diaphragm) as does glycosylation (di-, mono-, nonglycosylated; gastrocnemius, EDL, TA=60%, 30%, 10%; soleus, 30%, 40%, 30%; diaphragm, 30%, 30%, 40%). PrP is predominantly di-glycosylated in human diaphragm. PrP deficiency decreases body weight (15%) and EDL mass (9%); increases cytosolic oxidant activity (fiber bundles, 36%; C2C12 myotubes, 7%); and depresses specific force (12%) in adult (8-12 mos) but not adolescent (2 mos) mice. INNOVATION: This study is the first to directly assess a role of prion protein in skeletal muscle function.
conclusionsPrP content varies among murine skeletal muscles and is essential for maintaining normal redox homeostasis, muscle size, and contractile function in adult animals.
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