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Loss of Drosophila A-type lamin C initially causes tendon abnormality including disintegration of cytoskeleton and nuclear lamina in muscular defects
- Source :
-
Developmental Biology . Jan2013, Vol. 373 Issue 1, p216-227. 12p. - Publication Year :
- 2013
-
Abstract
- Abstract: Lamins are the major components of nuclear envelope architecture, being required for both the structural and informational roles of the nuclei. Mutations of lamins cause a spectrum of diseases in humans, including muscular dystrophy. We report here that the loss of the A-type lamin gene, lamin C in Drosophila resulted in pupal metamorphic lethality caused by tendon defects, matching the characteristics of human A-type lamin revealed by Emery–Dreifuss muscular dystrophy (EDMD). In tendon cells lacking lamin C activity, overall cell morphology was affected and organization of the spectraplakin family cytoskeletal protein Shortstop which is prominently expressed in tendon cells gradually disintegrated, notably around the nucleus and in a manner correlating well with the degradation of musculature. Furthermore, lamin C null mutants were efficiently rescued by restoring lamin C expression to shortstop-expressing cells, which include tendon cells but exclude skeletal muscle cells. Thus the critical function of A-type lamin C proteins in Drosophila musculature is to maintain proper function and morphology of tendon cells. [Copyright &y& Elsevier]
Details
- Language :
- English
- ISSN :
- 00121606
- Volume :
- 373
- Issue :
- 1
- Database :
- Academic Search Index
- Journal :
- Developmental Biology
- Publication Type :
- Academic Journal
- Accession number :
- 83576419
- Full Text :
- https://doi.org/10.1016/j.ydbio.2012.08.001