A-type lamin complexes and regenerative potential: a step towards understanding laminopathic diseases?
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.
Gotzmann
Josef
J
Max F. Perutz Laboratories, University Departments at the Vienna Biocenter, Department of Medical Biochemistry, Medical University of Vienna, Dr. Bohrgasse 9/3, 1030, Vienna, Austria.
Foisner
Roland
R
D000818:Animals; D002467:Cell Nucleus / Q000648:ultrastructure; D015870:Gene Expression / Q000502:physiology; D006801:Humans; D034904:Lamin Type A / Q000235:genetics / Q000502:physiology*; D012038:Regeneration / Q000235:genetics / Q000502:physiology*; D015398:Signal Transduction / Q000235:genetics / Q000502:physiology
D034904:Lamin Type A