Nucleotide excision repair disorders and the balance between cancer and aging

Jaan Olle Andressoo, Jan H.J. Hoeijmakers, James R. Mitchell

Research output: Contribution to journalReview articlepeer-review

70 Citations (Scopus)


Cancer incidence increases with age and is driven by accumulation of mutations in the DNA. In many so-called premature aging disorders, cancer appears earlier and at elevated rates. These diseases are predominantly caused by genome instability and present with symptoms, including cancer, resembling "segments" of aging and are thus often referred to as "segmental progerias". Two related segmental progerias, Cockayne syndrome (CS) and trichothiodystrophy (TTD), don't fit this pattern. Although caused by defects in genome maintenance via the nucleotide excision DNA repair (NER) pathway and displaying severe progeroid symptoms, CS and TTD patients appear to lack any cancer predisposition. More strikingly, genetic defects in the same NER pathway, and in some cases even within the same gene, XPD, can also give rise to disorders with greatly elevated cancer rates but without progeria (xeroderma pigmentosum). In this review, we will discuss the connection between genome maintenance, aging and cancer in light of a new mouse model of XPD disease.

Original languageEnglish
Pages (from-to)2886-2888
Number of pages3
JournalCell Cycle
Issue number24
Publication statusPublished - 15 Dec 2006
Externally publishedYes


  • Aging
  • Cancer
  • Cockayne syndrome
  • Insulin-like growth factor 1
  • Nucleotide excision repair
  • Xeroderma pigmentosum


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