Altered myogenesis in Six1-deficient mice

被引:210
作者
Laclef, C
Hamard, G
Demignon, J
Souil, E
Houbron, C
Maire, P
机构
[1] Univ Paris 05, Dept Genet Dev & Pathol Mol, Inst Cochin, INSERM 567,CNRS,UMR 8104, F-75014 Paris, France
[2] Univ Paris 05, Plateforme Recombinaison Homologue, Inst Cochin, INSERM 567,CNRS,UMR 8104, F-75014 Paris, France
[3] Univ Paris 05, Plateforme Histol, Inst Cochin, INSERM 567,CNRS,UMR 8104, F-75014 Paris, France
来源
DEVELOPMENT | 2003年 / 130卷 / 10期
关键词
Six/sine oculis homeoproteins; myogenesis; MyoD; myogenin; Myf5; Pax3;
D O I
10.1242/dev.00440
中图分类号
Q [生物科学];
学科分类号
07 ; 0710 ; 09 ;
摘要
Six homeoproteins are expressed in several tissues, including muscle, during vertebrate embryogenesis, suggesting that they may be involved in diverse differentiation processes. To determine the functions of the Six1 gene during myogenesis, we constructed Six1-deficient mice by replacing its first exon with the lacZ gene. Mice lacking Six1 die at birth because of severe rib malformations and show extensive muscle hypoplasia affecting most of the body muscles in particular certain hypaxial muscles. Six1(-/-) embryos have impaired primary myogenesis, characterized, at E13.5, by a severe reduction and disorganisation of primary myofibers in most body muscles. While Myf5, MyoD and myogenin are correctly expressed in the somitic compartment in early Six1(-/-) embryos, by E11.5 MyoD and myogenin gene activation is reduced and delayed in limb buds. However, this is not the consequence of a reduced ability of myogenic precursor cells to migrate into the limb buds or of an abnormal apoptosis of myoblasts lacking Six1. It appears therefore that Six1 plays a specific role in hypaxial muscle differentiation, distinct from those of other hypaxial determinants such as Pax3, cMet, Lbx1 or Mox2.
引用
收藏
页码:2239 / 2252
页数:14
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