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dc.contributor.authorCorrera, Rosa Maria
dc.contributor.authorOllitrault, David
dc.contributor.authorValente, Mariana
dc.contributor.authorMazzola, Alessia
dc.contributor.authorAdalsteinsson, Bjorn T
dc.contributor.authorFerguson-Smith, Anne C
dc.contributor.authorMarazzi, Giovanna
dc.contributor.authorSassoon, David A
dc.date.accessioned2018-11-22T00:33:18Z
dc.date.available2018-11-22T00:33:18Z
dc.date.issued2018-10-02
dc.identifier.issn2045-2322
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/285694
dc.description.abstractPw1/Peg3 is an imprinted gene expressed from the paternally inherited allele. Several imprinted genes, including Pw1/Peg3, have been shown to regulate overall body size and play a role in adult stem cells. Pw1/Peg3 is expressed in muscle stem cells (satellite cells) as well as a progenitor subset of muscle interstitial cells (PICs) in adult skeletal muscle. We therefore examined the impact of loss-of-function of Pw1/Peg3 during skeletal muscle growth and in muscle stem cell behavior. We found that constitutive loss of Pw1/Peg3 function leads to a reduced muscle mass and myofiber number. In newborn mice, the reduction in fiber number is increased in homozygous mutants as compared to the deletion of only the paternal Pw1/Peg3 allele, indicating that the maternal allele is developmentally functional. Constitutive and a satellite cell-specific deletion of Pw1/Peg3, revealed impaired muscle regeneration and a reduced capacity of satellite cells for self-renewal. RNA sequencing analyses revealed a deregulation of genes that control mitochondrial function. Consistent with these observations, Pw1/Peg3 mutant satellite cells displayed increased mitochondrial activity coupled with accelerated proliferation and differentiation. Our data show that Pw1/Peg3 regulates muscle fiber number determination during fetal development in a gene-dosage manner and regulates satellite cell metabolism in the adult.
dc.format.mediumElectronic
dc.languageeng
dc.publisherSpringer Science and Business Media LLC
dc.rightsAttribution 4.0 International
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/
dc.subjectCells, Cultured
dc.subjectSatellite Cells, Skeletal Muscle
dc.subjectAnimals
dc.subjectAnimals, Newborn
dc.subjectMice, Transgenic
dc.subjectMice
dc.subjectModels, Animal
dc.subjectRegeneration
dc.subjectGenomic Imprinting
dc.subjectFetal Development
dc.subjectMuscle Development
dc.subjectGene Dosage
dc.subjectMale
dc.subjectKruppel-Like Transcription Factors
dc.subjectMuscle Fibers, Skeletal
dc.subjectCell Self Renewal
dc.titleThe imprinted gene Pw1/Peg3 regulates skeletal muscle growth, satellite cell metabolic state, and self-renewal.
dc.typeArticle
prism.issueIdentifier1
prism.publicationDate2018
prism.publicationNameSci Rep
prism.startingPage14649
prism.volume8
dc.identifier.doi10.17863/CAM.33044
dcterms.dateAccepted2018-09-18
rioxxterms.versionofrecord10.1038/s41598-018-32941-x
rioxxterms.licenseref.urihttp://www.rioxx.net/licenses/all-rights-reserved
rioxxterms.licenseref.startdate2018-10-02
dc.contributor.orcidAdalsteinsson, Bjorn T [0000-0001-9777-1714]
dc.contributor.orcidSassoon, David A [0000-0001-6074-048X]
dc.identifier.eissn2045-2322
rioxxterms.typeJournal Article/Review
pubs.funder-project-idMedical Research Council (MR/R009791/1)
cam.issuedOnline2018-10-02


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Attribution 4.0 International
Except where otherwise noted, this item's licence is described as Attribution 4.0 International