X-Message-Number: 29186
Date: Sat, 24 Feb 2007 20:16:42 -0800 (PST)
From: 
Subject: the shortest mammalian lifespan

[Its hard to believe that there exists a progeroid mouse strain that lives
for just 4 weeks. It wouldn't take very long to test out life extending
interventions in this rodent strain...]

Nature. 2003 May 15;423(6937):298-301.
A progeroid syndrome in mice is caused by defects in A-type
lamins.Mounkes LC, Kozlov S, Hernandez L, Sullivan T, Stewart CL.  Cancer
and Developmental Biology Laboratory, National Cancer Institute,
Frederick, Maryland 21702, USA.
  Numerous studies of the underlying causes of ageing have been attempted
by examining diseases associated with premature ageing, such as Werner's
syndrome and Hutchinson-Gilford progeria syndrome (HGPS). HGPS is a rare
genetic disorder resulting in phenotypes suggestive of accelerated
ageing, including shortened stature, craniofacial disproportion, very thin
skin, alopecia and osteoporosis, with death in the early teens
predominantly due to atherosclerosis. However, recent reports suggest
that developmental abnormalities may also be important in HGPS. Here we
describe the derivation of mice carrying an autosomal recessive mutation
in the lamin A gene (Lmna) encoding A-type lamins, major components of
the nuclear lamina. Homozygous mice display defects consistent with HGPS,
including a marked reduction in growth rate and death by 4 weeks of
age. Pathologies in bone, muscle and skin are also consistent with
progeria. The Lmna mutation resulted in nuclear morphology defects and
decreased lifespan of homozygous fibroblasts, suggesting premature cell
death. Here we present a mouse model for progeria that may elucidate
mechanisms of ageing and development in certain tissue types,
especially those developing from the mesenchymal cell lineage.
PMID: 12748643

Rate This Message: http://www.cryonet.org/cgi-bin/rate.cgi?msg=29186