By investigating a consanguineous family with 4 affected children carrying a homozygous LMNA mutation (c.1626G>C; p.K542N) we previously provided evidence for an autosomal recessive form of HGPS, thus challenging the prevailing view that HGPS merely represents a sporadic autosomal dominant, lamin A-related laminopathy. This evidence concerns the gene LMNA and laminopathy.