A-type lamin complexes and regenerative potential: a step towards understanding laminopathic diseases?

被引：86

作者：

Gotzmann, J ^{[1
]}

Foisner, R ^{[1
]}

机构：

[1] Med Univ Vienna, Univ Dept Vienna Bioctr, Dept Biochem Med, Max F Perutz Labs, A-1030 Vienna, Austria

来源：

HISTOCHEMISTRY AND CELL BIOLOGY | 2006年 / 125卷 / 1-2期

基金：

奥地利科学基金会;

关键词：

cell cycle; chromatin organization; lamina-associated proteins; laminopathies; lamins; LEM-domain; nuclear architecture; nuclear envelope; stem cells; tissue regeneration;

D O I：

10.1007/s00418-005-0050-8

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

The lamins are nuclear intermediate filament-type proteins forming the nuclear lamina meshwork at the inner nuclear membrane as well as complexes in the nucleoplasm. The recent discoveries that mutated A-type lamins and lamin-binding nuclear membrane proteins can be linked to numerous rare human diseases (laminopathies) affecting a multitude of tissues has changed the cell biologist's view of lamins as mere structural nuclear scaffold proteins. It is still unclear how mutations in these ubiquitously expressed proteins give rise to tissue-restricted pathological phenotypes. Potential disease models include mutation-caused defects in lamin structure and stability, the deregulation of gene expression, and impaired cell cycle control. This review brings together various previously proposed ideas and suggests a novel, more general, disease model based on an impairment of adult stem cell function and thus compromised tissue regeneration in laminopathic diseases.

引用

页码：33 / 41

页数：9

共 75 条

[1] Mouse model carrying H222P-Lmna mutation develops muscular dystrophy and dilated cardiomyopathy similar to human striated muscle laminopathies [J].