Matches in SemOpenAlex for { <https://semopenalex.org/work/W2013627478> ?p ?o ?g. }
Showing items 1 to 78 of
78
with 100 items per page.
- W2013627478 endingPage "750" @default.
- W2013627478 startingPage "750" @default.
- W2013627478 abstract "Limb Girdle Muscular Dystrophy Type 2B (LGMD2B) and Miyoshi Myopathy (MM) are caused by mutations in the dysferlin gene, but the role of dysferlin in healthy muscle and the changes that occur when it is mutated or absent are poorly understood. Previous work supported a role for dysferlin in sarcolemmal repair following laser wounding or other in vitro injuries. We study the response of A/J mice, which lack dysferlin, to injury by large-strain lengthening contractions in vivo. We find that dysferlin promotes normal recovery from this physiological injury but is not necessary for sarcolemmal repair. Consistent with this, immunofluorescence microscopic studies of healthy muscle, that we fixed and treated in a hot, mildly acidic solution to expose dysferlin’s epitopes, show that dysferlin is primarily in transverse tubules (TT), not the sarcolemma as previously reported. Furthermore, TT are disrupted when skeletal muscle is injured physiologically, and disruption is much more extensive in A/J muscles than in controls. Studies of FDB myofibers in tissue culture also demonstrate the presence of dysferlin in TT. Brief exposure of control myofibers to hypoosmotic solutions damages TT, in a process dependent upon extracellular Ca2+. As in vivo, A/J myofibers are more extensively damaged by osmotic shock than controls; they are indistinguishable from controls when shocked in Ca2+-free medium, however. Thus Ca2+ may promote damage, rather than participate in dysferlin-dependent membrane repair, as previously reported. Our results suggest that the changes in TT following injury in vivo and in vitro are similar, that they require extracellular Ca2+, and that they are much more pronounced when dysferlin is absent. We propose that dysferlin is essential for the integrity of the TT of skeletal muscle, in maintaining this integrity during contraction and relaxation, and in repairing damaged TT following injury. Supported by the Jain Foundation." @default.
- W2013627478 created "2016-06-24" @default.
- W2013627478 creator A5012466372 @default.
- W2013627478 creator A5018365166 @default.
- W2013627478 creator A5035163781 @default.
- W2013627478 creator A5040198794 @default.
- W2013627478 creator A5042232757 @default.
- W2013627478 creator A5081683404 @default.
- W2013627478 date "2011-10-01" @default.
- W2013627478 modified "2023-10-16" @default.
- W2013627478 title "O.21 Cellular and molecular mechanisms of dysferlinopathy: New insights" @default.
- W2013627478 doi "https://doi.org/10.1016/j.nmd.2011.06.1115" @default.
- W2013627478 hasPublicationYear "2011" @default.
- W2013627478 type Work @default.
- W2013627478 sameAs 2013627478 @default.
- W2013627478 citedByCount "0" @default.
- W2013627478 crossrefType "journal-article" @default.
- W2013627478 hasAuthorship W2013627478A5012466372 @default.
- W2013627478 hasAuthorship W2013627478A5018365166 @default.
- W2013627478 hasAuthorship W2013627478A5035163781 @default.
- W2013627478 hasAuthorship W2013627478A5040198794 @default.
- W2013627478 hasAuthorship W2013627478A5042232757 @default.
- W2013627478 hasAuthorship W2013627478A5081683404 @default.
- W2013627478 hasConcept C104317684 @default.
- W2013627478 hasConcept C105702510 @default.
- W2013627478 hasConcept C185592680 @default.
- W2013627478 hasConcept C199096232 @default.
- W2013627478 hasConcept C207001950 @default.
- W2013627478 hasConcept C207200792 @default.
- W2013627478 hasConcept C2778649560 @default.
- W2013627478 hasConcept C2779030066 @default.
- W2013627478 hasConcept C2779959927 @default.
- W2013627478 hasConcept C28406088 @default.
- W2013627478 hasConcept C37113945 @default.
- W2013627478 hasConcept C501734568 @default.
- W2013627478 hasConcept C54355233 @default.
- W2013627478 hasConcept C55493867 @default.
- W2013627478 hasConcept C68731436 @default.
- W2013627478 hasConcept C86803240 @default.
- W2013627478 hasConcept C95444343 @default.
- W2013627478 hasConceptScore W2013627478C104317684 @default.
- W2013627478 hasConceptScore W2013627478C105702510 @default.
- W2013627478 hasConceptScore W2013627478C185592680 @default.
- W2013627478 hasConceptScore W2013627478C199096232 @default.
- W2013627478 hasConceptScore W2013627478C207001950 @default.
- W2013627478 hasConceptScore W2013627478C207200792 @default.
- W2013627478 hasConceptScore W2013627478C2778649560 @default.
- W2013627478 hasConceptScore W2013627478C2779030066 @default.
- W2013627478 hasConceptScore W2013627478C2779959927 @default.
- W2013627478 hasConceptScore W2013627478C28406088 @default.
- W2013627478 hasConceptScore W2013627478C37113945 @default.
- W2013627478 hasConceptScore W2013627478C501734568 @default.
- W2013627478 hasConceptScore W2013627478C54355233 @default.
- W2013627478 hasConceptScore W2013627478C55493867 @default.
- W2013627478 hasConceptScore W2013627478C68731436 @default.
- W2013627478 hasConceptScore W2013627478C86803240 @default.
- W2013627478 hasConceptScore W2013627478C95444343 @default.
- W2013627478 hasIssue "9-10" @default.
- W2013627478 hasLocation W20136274781 @default.
- W2013627478 hasOpenAccess W2013627478 @default.
- W2013627478 hasPrimaryLocation W20136274781 @default.
- W2013627478 hasRelatedWork W115279680 @default.
- W2013627478 hasRelatedWork W2015163805 @default.
- W2013627478 hasRelatedWork W2043172254 @default.
- W2013627478 hasRelatedWork W2047136624 @default.
- W2013627478 hasRelatedWork W2066870043 @default.
- W2013627478 hasRelatedWork W2145659187 @default.
- W2013627478 hasRelatedWork W2158835728 @default.
- W2013627478 hasRelatedWork W2167813635 @default.
- W2013627478 hasRelatedWork W3163208405 @default.
- W2013627478 hasRelatedWork W4251516726 @default.
- W2013627478 hasVolume "21" @default.
- W2013627478 isParatext "false" @default.
- W2013627478 isRetracted "false" @default.
- W2013627478 magId "2013627478" @default.
- W2013627478 workType "article" @default.