Article
Hutchinson-Gilford progeria syndrome: oral and craniofacial phenotypes.
Oral diseases - 1 Apr 2009
Domingo D L, Trujillo M I, Council S E, Merideth M A, Gordon L B, Wu T, Introne W J, Gahl W A, Hart T C
Abstract excerpt
OBJECTIVE: Hutchinson-Gilford progeria syndrome (HGPS) is a rare early-onset accelerated senescence syndrome. In HGPS, a recently identified de novo dominant mutation of the lamin A gene (LMNA) produces abnormal lamin A, resulting in compromised nuclear membrane integrity. Clinical features include sclerotic skin, cardiovascular and bone abnormalities, and marked growth retardation. Craniofacial features include...
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