(WS) is a rare autosomal recessive disease characterized by premature aging, which was first described by Werner in 1904 .
Vitamin C alleviates aging defects in a stem cell model for Werner syndrome
. Protein Cell.
Lebel et al., "Mitochondrial dysfunction in some oxidative stress-related genetic diseases: ataxia-Telangiectasia, Down Syndrome, Fanconi Anaemia and Werner Syndrome
," Biogerontology, vol.
is a genetic disorder that causes people to age more rapidly than normal.
It is caused by null mutations at WRN locus, which codes for a member of RecQ family of DNA helicases.6-9,11 The disease is associated with excessive synthesis of collagen type I and III which is dependent on elevated messenger RNA(mRNA) levels.6 The locus of Werner Syndrome
has been found on the short arm of chromosome 8 in both Japanese and non Japanes.5,7,11 Fibroblasts isolated from WS patients exhibit genomic instability, increased sensitivity to specific DNA damaging agents, slow proliferation, lengthened S-phase, and accelerated replicative senescence.5,7,16
Furthermore, a number of inherited conditions, for example Down syndrome, Fanconi anemia, Bloom syndrome, Wiskott-Aldrich syndrome, dyskeratosis congenita, Werner syndrome
, Shwachman syndrome, Blackfan-Diamond syndrome, and Klinefelter syndrome, carry an increased risk of AML [5,6].
Flies with damage to the gene share key features with people afflicted by the rapid ageing condition Werner syndrome
. In particular, the flies have unstable DNA and their chromosomes are often altered.
Flies with damaged genes share key features with people hit by rapid ageing condition Werner syndrome
. Oxford University's Dr Lynne Cox said it will "help us understand normal ageing" - increasingly seen by scientists as important with an ageing population.
A small handful of medical conditions are associated with premature graying, one example being Werner Syndrome
, a rare disorder characterized by an accelerated rate of aging following the onset of puberty.
The NIEHS has made a similar investment in projects investigating diseases that cause premature aging, such as Werner syndrome
. "All of these inherited diseases have allowed us to understand pathways ...