Please use this identifier to cite or link to this item: http://hdl.handle.net/1822/29611

TitleAtaxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levels
Author(s)Costa, Maria do Carmo
Bajanca, Fernanda
Rodrigues, Ana João
Tomé, Ricardo J.
Corthals, Gary
Ribeiro, Sandra Macedo
Paulson, Henry L.
Logarinho, Elsa
Maciel, P.
Issue dateJul-2010
PublisherPLOS
JournalPLoS ONE
Abstract(s)BACKGROUND: During myogenesis several transcription factors and regulators of protein synthesis and assembly are rapidly degraded by the ubiquitin-proteasome system (UPS). Given the potential role of the deubiquitinating enzyme (DUB) ataxin-3 in the UPS, and the high expression of the murine ataxin-3 homolog in muscle during embryogenesis, we sought to define its role in muscle differentiation. METHODOLOGY/PRINCIPAL FINDINGS: Using immunofluorescence analysis, we found murine ataxin-3 (mATX3) to be highly expressed in the differentiated myotome of E9.5 mouse embryos. C2C12 myoblasts depleted of mATX3 by RNA interference exhibited a round morphology, cell misalignment, and a delay in differentiation following myogenesis induction. Interestingly, these cells showed a down-regulation of alpha5 and alpha7 integrin subunit levels both by immunoblotting and immunofluorescence. Mouse ATX3 was found to interact with alpha5 integrin subunit and to stabilize this protein by repressing its degradation through the UPS. Proteomic analysis of mATX3-depleted C2C12 cells revealed alteration of the levels of several proteins related to integrin signaling. CONCLUSIONS: Ataxin-3 is important for myogenesis through regulation of integrin subunit levels.
Typearticle
URIhttp://hdl.handle.net/1822/29611
DOI10.1371/journal.pone.0011728
ISSN1932-6203
Publisher versionhttp://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0011728
Peer-Reviewedyes
AccessopenAccess
Appears in Collections:ICVS - Artigos em Revistas Internacionais com Referee

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