Increased expression of the Huntingtin interacting protein-1 gene in cells from Hutchinson Gilford Syndrome (Progeria) patients and aged donors

J Gerontol A Biol Sci Med Sci. 2003 Oct;58(10):B873-8. doi: 10.1093/gerona/58.10.b873.

Abstract

Hutchinson Gilford syndrome (progeria [PG]) is a human disease associated with accelerated aging. To elucidate the acceleration mechanism, we first tried to transform a PG-derived cell line by infection of a recombinant adenovirus expressing HPV (human papilloma virus)-E6 and HPV-E7 genes. The transfected PG cells had a greater number of population doublings (PD) (>80), faster doubling time, and less staining of senescence-associated ss-galactosidase than the nontransfected PG cells. The transfected cells also showed markedly more detectable telomerase activity than the nontransformed cells. The expression levels of the genes in the E6-transduced and E7-transduced cell line were then compared with those of the nontransfected cell line using an mRNA differential display method, following reverse-transcriptase polymerase chain reaction analysis. Expression of huntingtin interacting protein-1 (HIP-1) gene was found to be increased not only in PG cells but also in fibroblast cells from aged healthy donors. Thus, HIP-1 might be a molecular assistant in the pathogenesis of the cellular senescent process in the human cells tested.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Aged
  • Carrier Proteins / genetics*
  • Cell Line
  • Cells, Cultured
  • Cellular Senescence
  • Child
  • DNA-Binding Proteins*
  • Fibroblasts
  • Gene Expression Regulation*
  • Humans
  • Papillomaviridae
  • Progeria / genetics*
  • RNA, Messenger / genetics
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Analysis, DNA
  • Telomerase / metabolism
  • Transcription, Genetic
  • beta-Galactosidase / metabolism

Substances

  • Carrier Proteins
  • DNA-Binding Proteins
  • HIP1 protein, human
  • RNA, Messenger
  • Telomerase
  • beta-Galactosidase