HISTOLOGY AND HISTOPATHOLOGY

Cellular and Molecular Biology

 

Review

Lamin A/C, laminopathies and premature ageing

Baohua Liu and Zhongjun Zhou

Department of Biochemistry, The University of Hong Kong, Pokfulam, Hong Kong.

Offprint requests to: Zhongjun Zhou, Department of Biochemistry, The Univeristy of Hong Kong, 31 Sassoon Road, Pookfulam, Hong Kong. e-mail: zhongjun@hkucc.hku.hk


Summary. Lamin A/C belongs to type V intermediate filaments and constitutes the nuclear lamina and nuclear matrix, where a variety of nuclear activities occur. Lamin A/C protein is firstly synthesized as a precursor and is further proteolytically processed by the zinc metallo-proteinase Ste24 (Zmpste24). Lamin A/C mutations cause a series of human diseases, collectively called laminopathies, the most severe of which is Hutchinson Gilford progeria syndrome (HGPS) and restrictive dermopathy (RD) which arises due to an unsuccessful maturation of prelamin A. Although the exact underlying molecular mechanisms are still poorly understood, genomic instability, defective nuclear mechanics and mechanotransduction, have been hypothesized to be responsible for laminopathy-based premature ageing. Removal of unprocessed prelamin A (progerin) or rescue of defective DNA repair could be potential therapeutic strategies for the treatment of HGPS in future
. Histol Histopathol 23, 747-763 (2008)

Key words: Lamin A/C, Laminopathy, Premature ageing