Cardiovascular lesions and skeletal myopathy in mice lacking desmin.

Abstract:

:In order to further our understanding of the biological role of desmin, the muscle-specific intermediate filament protein, a null mutation in the desmin gene was introduced into the germ line of mice. Despite the complete lack of desmin, these mice developed and reproduced. Since we show that skeletal, cardiac, and smooth muscles form in the Des-/- mice, it is reasonable to propose that desmin is not essential for myogenic commitment or for myoblast fusion or differentiation in vivo. However, morphological abnormalities were observed in the diaphragm of adult mice; these were demonstrated by disorganized, distended, and nonaligned fibers. The heart presented areas of hemorrhaging in which fibrosis and ischemia were observed. We have also shown that the absence of desmin produces specific defects in smooth muscles. In conclusion, our results have demonstrated that desmin is not required for the differentiation of skeletal, cardiac, and smooth muscles but is essential to strengthen and maintain the integrity of these tissues.

journal_name

Dev Biol

journal_title

Developmental biology

authors

Li Z,Colucci-Guyon E,Pinçon-Raymond M,Mericskay M,Pournin S,Paulin D,Babinet C

doi

10.1006/dbio.1996.0122

subject

Has Abstract

pub_date

1996-05-01 00:00:00

pages

362-6

issue

2

eissn

0012-1606

issn

1095-564X

pii

S0012-1606(96)90122-0

journal_volume

175

pub_type

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