A-type lamins: guardians of the soma?
The gene LMNA encodes the proteins lamins A and C and is implicated in nine different laminopathies - inherited diseases that are linked to premature ageing. Recent evidence has demonstrated that lamins A and C have essential functions in protecting cells from physical damage, as well as in maintaining the function of transcription factors required for the differentiation of adult stem cells. Thus, the degenerative nature of laminopathies is explained because these lamins are essential for maintenance of somatic tissues in adulthood.
Hutchison
Chris J
CJ
School of Biological and Biomedical Sciences, The University of Durham, South Road, Durham, DH1 4EB, UK. c.j.hutchison@durham.ac.uk
Worman
Howard J
HJ
D015870:Gene Expression; D030342:Genetic Diseases, Inborn / Q000235:genetics; D006801:Humans; D034882:Lamins / Q000235:genetics / Q000502:physiology*; D009154:Mutation; D014158:Transcription, Genetic / Q000502:physiology
D034882:Lamins