Review

Cellular and Molecular Life Sciences

, Volume 64, Issue 2, pp 155-170

Human progeroid syndromes, aging and cancer: new genetic and epigenetic insights into old questions

  • C. L. RamírezAffiliated withDepartamento de Bioquímica y Biología Molecular, Facultad de Medicina, Instituto Universitario de Oncología, Universidad de Oviedo
  • , J. CadiñanosAffiliated withDepartamento de Bioquímica y Biología Molecular, Facultad de Medicina, Instituto Universitario de Oncología, Universidad de Oviedo
  • , I. VarelaAffiliated withDepartamento de Bioquímica y Biología Molecular, Facultad de Medicina, Instituto Universitario de Oncología, Universidad de Oviedo
  • , J. M. P. FreijeAffiliated withDepartamento de Bioquímica y Biología Molecular, Facultad de Medicina, Instituto Universitario de Oncología, Universidad de Oviedo
  • , C. López-OtínAffiliated withDepartamento de Bioquímica y Biología Molecular, Facultad de Medicina, Instituto Universitario de Oncología, Universidad de Oviedo Email author 

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Abstract.

Disorders in which individuals exhibit certain features of aging early in life are referred to as segmental progeroid syndromes. With the progress that has been made in understanding the etiologies of these conditions in the past decade, potential therapeutic options have begun to move from the realm of improbability to initial stages of testing. Among these syndromes, relevant advances have recently been made in Werner syndrome, one of several progeroid syndromes characterized by defective DNA helicases, and Hutchinson-Gilford progeria syndrome, which is characterized by aberrant processing of the nuclear envelope protein lamin A. Although best known for their causative roles in these illnesses, Werner protein and lamin A have also recently emerged as key players vulnerable to epigenetic changes that contribute to tumorigenesis and aging. These advances further demonstrate that understanding progeroid syndromes and introducing adequate treatments will not only prove beneficial to patients suffering from these dramatic diseases, but will also provide new mechanistic insights into cancer and normal aging processes.

Keywords.

Accelerated aging progeria cancer Bloom’s syndrome nuclear envelope lamin A metalloproteinase Werner syndrome DNA repair farnesyl transferase inhibitor ataxia telangiectasia