History of EuroProgeria

Progeria is a rare, genetically determined condition characterized by accelerated aging in children. Its name is derived from Greek and means "prematurely old". The classic type is the Hutchinson-Gilford Progeria Syndrome (HGPS) which was first described in England in 1886 by Dr. Jonathan Hutchinson and again in 1886 by Dr. Hastings Gilford. In early 2003 two working groups (French and American, respectively) succeeded in identifying point mutations in the lamin A/C gene as the cause of HGPS.

 HGPS is the most recent and most conspicuous disorder added to the expanding catalogue of 'laminopathies', diseases caused by mutations affecting nuclear lamina proteins. The (eight) disorders associated with mutations in LMNA are diverse, both in symptomatology and in pattern of inheritance. Dominant disorders are Dilated Cardiomyopathy with conduction defect, Familial Partial Lipodystrophy, Emery-Dreifuss Muscular Dystrophy (AD-EDMD or EMD2), Limb-Girdle Muscular Dystrophy with conduction defects, and Hutchinson-Gilford Progeria. Recessive disorders are Emery-Dreifuss Muscular Dystrophy (AR-EDMD or EMD3), Charcot-Marie-Tooth Disease type 2 and Mandibuloacral Dysplasia.

 The third European reunion of families with a child with progeria, gathered within the European Progeria Family Circle, was held from 25^th September - 29^th September 2003 in Magdeburg, Germany. The first European symposium, aimed at presenting a clinical and molecular update of HGPS, was scheduled to coincide with this. During this symposium, a number of European experts in the field of laminopathies as well as clinicians with experience in the diagnosis and treatment of progeria and/or progeria-like syndromes and in relevant research presented their experiences. A decision was also made by the participants to work closely together in the field of progeria research. As a consequence, EuroProgeria, the European network for the scientific investigation of Hutchinson-Gilford Progeria, was established.