Premature aging syndromes represent human conditions in which multiple tissues and organs show features of accelerated aging. This reference module provides un up to date review of Hutchinson-Gilford Progeria Syndrome (HGPS) and Werner Syndrome (WS), two of the best-characterized segmental progeroid disorders in humans, which are also named childhood-progeria and adulthood-progeria, respectively. A description is provided of their main symptoms, genetic causes, molecular pathology, and therapeutic approaches, alongside with a list of online disease registers and databases. Furthermore, attention is paid to atypical progeroid syndromes (atypical HGPS or atypical WS), a spectrum of progeroid disease phenotypes ranging from moderate and mild-severe to very aggressive forms and often caused by less frequent mutations in the same genes causing typical premature aging syndromes.
Premature aging syndromes
Coppede F.
Primo
2018-01-01
Abstract
Premature aging syndromes represent human conditions in which multiple tissues and organs show features of accelerated aging. This reference module provides un up to date review of Hutchinson-Gilford Progeria Syndrome (HGPS) and Werner Syndrome (WS), two of the best-characterized segmental progeroid disorders in humans, which are also named childhood-progeria and adulthood-progeria, respectively. A description is provided of their main symptoms, genetic causes, molecular pathology, and therapeutic approaches, alongside with a list of online disease registers and databases. Furthermore, attention is paid to atypical progeroid syndromes (atypical HGPS or atypical WS), a spectrum of progeroid disease phenotypes ranging from moderate and mild-severe to very aggressive forms and often caused by less frequent mutations in the same genes causing typical premature aging syndromes.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.