Progeroid syndromes

From Simple English Wikipedia, the free encyclopedia

Progeroid syndromes (PS) describes a range of genetic disorders where the affected person appears to grow older faster.[1] All these disorders are monogenetic,[2] meaning they come from mutations of a single gene. Most known PS mutations lead to either defects in the DNA repair mechanism, or defects in a protein known as lamin A/C.

Progeroid means "resembling old age". This definition can be applied to many different diseases. Alzheimer's disease and Parkinson's disease affects only one tissue. In most cases, the term progeroid syndrome is used for cases where the affected people only show some of the features of aging, but not all of them. In these cases, many different kinds of tissue are affected.

Individuals with PS-related disorders often have a reduced lifespan.[2] The most widely studied of the progeroid syndromes are Werner syndrome (WS) and Hutchinson-Gilford Progeria Syndrome (HGPS), because they resemble natural aging.[2]

Because of its property of accelerated aging (senescence), progeroid syndomes have been widely studied in the fields of aging,regeneration, stem cells and cancer.[2]

References[change | change source]

  1. Navarro, Claire L.; Cau, Pierre; Lévy, Nicolas (2006). "Molecular bases of progeroid syndromes". Human Molecular Genetics. 15: R151–R161. doi:10.1093/hmg/ddl214. PMID 16987878.
  2. 2.0 2.1 2.2 2.3 Navarro, CL; Cau, P; Lévy, N (2006). "Molecular bases of progeroid syndromes". Human Molecular Genetics. 15 Spec No 2: R151–61. doi:10.1093/hmg/ddl214. PMID 16987878.