These cells could be grown in vitro for a long time as non-immortalized cell lines and differentiate also toward neurons and glia cells. She concluded that cultured lines of these stem cells could provide a valuable authologous material for transplantation to patients that present with progeria. Role of lamins in chromatin organization R. Foisner presented his studies aimed at clarifying the role of nucleoplasmic lamins in chromatin organization Inhibitors,research,lifescience,medical and possible implications for laminopathies

(17). He has identified a nucleoplasmic A-type lamin-binding protein, termed Lamin-associated Polypeptide 2 alpha (LAP2α) (18), which impairs assembly of A-type lamins at the nuclear lamina and maintains a pool of soluble, mobile A-type lamins throughout the nucleus. He also showed Inhibitors,research,lifescience,medical that a nucleoplasmic complex of A-type lamins and LAP2α increases the repressor activity of the cell cycle regulatory retinoblastoma protein (pRb). Furthermore the deletion of the Lap2α gene in mice causes loss of nucleoplasmic lamins and a deregulation of pRb-mediated gene expression, leading to hyperproliferation of tissue progenitor cells and hyperplasia of the tissue (18). He proposed a model in which a nucleoplasmic Inhibitors,research,lifescience,medical pool of lamins is involved in the regulation of chromatin structure and function in tissue progenitor cells during tissue

regeneration; he postulated that mutations in lamins can

alter the Inhibitors,research,lifescience,medical ratio of nucleoplasmic versus peripheral lamins and thereby affect tissue progenitor cells and tissue regeneration. Role of mutated lamin A and emerin proteins in development of abnormal phenotypes and prospects for gene therapy This particular aspect of lamins was illustrated by R. Rzepecki. Mutations in LMNA and STA genes affect major cellular pathways regulating the development, maintenance and regeneration of tissues, mostly cardiac and skeletal muscles, of mesodermal origin. Lamin A, lamin B, emerin, Inhibitors,research,lifescience,medical NET25, NET39 and MAN1 (LEMD3) proteins modulate such signaling almost pathways e.g.: Wnt, TGFβ/ BMP/activin, MAPKs, mTOR, Akt, PKC (19). Most of these pathways interconnect themselves and with many other pathways giving rise to the differences in manifestations of disease phenotypes. Preliminary reports demonstrate the possibility to use gene/cell therapy for the muscular dystrophy type of laminopathies as well as for HGPS Progeria. Strategies for gene therapy for AR type of laminopathies seem to be the simplest, while the prospect gene therapy treatment of AD laminopathies seems mutato be much more complicated (20). Lentivirus vector system for Selleckchem Silmitasertib delivery of genetic drug represents a model of universal gene therapy strategy for muscle laminopathies and HGPS progeria. Clinical aspects of laminopathies The second day was opened by G.