Premature aging syndromes are human conditions in which multiple organs and tissues show features of accelerated aging. This chapter describes Hutchinson–Gilford Progeria Syndrome and Werner Syndrome, two of the best-characterized segmental progeroid disorders in humans, and provides a summary of their main symptoms, genetic causes, molecular pathology, and therapeutic approaches. Furthermore, attention is paid to available cell culture models, including induced pluripotent stem cells and mouse models of both disorders, which represent invaluable tools for the investigation of their molecular mechanisms and for testing the effects of potential therapeutic compounds
Premature Aging Syndrome
Coppedè, Fabio
Primo
2018-01-01
Abstract
Premature aging syndromes are human conditions in which multiple organs and tissues show features of accelerated aging. This chapter describes Hutchinson–Gilford Progeria Syndrome and Werner Syndrome, two of the best-characterized segmental progeroid disorders in humans, and provides a summary of their main symptoms, genetic causes, molecular pathology, and therapeutic approaches. Furthermore, attention is paid to available cell culture models, including induced pluripotent stem cells and mouse models of both disorders, which represent invaluable tools for the investigation of their molecular mechanisms and for testing the effects of potential therapeutic compoundsI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
