Laminopathy is a heterogeneous group of inherited disorders resulting from abnormalities of type A lamins. Progeroid laminopathy is a group of the laminopathies that mimics clinical and molecular features of aging. It can be caused by mutations in the A-type lamin gene (LMNA), altering prelamin A itself, or in ZMPSTE24, encoding an endoprotease involved in its processing. Apart from some atypical progeroid forms there are three major syndromes: Hutchinson Gilford progeria syndrome [DS:H00601], Restrictive dermopathy [DS:H00665], Mandibuloacral dysplasia [DS:H00663].
