Loss of Drosophila A-type lamin C initially causes tendon abnormality including disintegration of cytoskeleton and nuclear lamina in muscular defects.
Dev Biol
; 373(1): 216-27, 2013 Jan 01.
Article
em En
| MEDLINE
| ID: mdl-22982669
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Tendões
/
Citoesqueleto
/
Proteínas de Drosophila
/
Lâmina Nuclear
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Lamina Tipo A
/
Drosophila
/
Proteínas dos Microfilamentos
Tipo de estudo:
Etiology_studies
Limite:
Animals
Idioma:
En
Revista:
Dev Biol
Ano de publicação:
2013
Tipo de documento:
Article
País de afiliação:
Japão