Journal
BIOCHEMICAL SOCIETY TRANSACTIONS
Volume 36, Issue -, Pages 1389-1392Publisher
PORTLAND PRESS LTD
DOI: 10.1042/BST0361389
Keywords
Drosophila melanogaster; Hutchinson-Gilford progeria syndrome (HGPS); lamin A; nuclear structure; progerin
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The laminopathy Hutchinson-Gilford progeria syndrome (HGPS) is caused by the mutant lamin A protein progerin and leads to premature aging of affected children. Despite numerous cell biological and biochemical insights into the basis for the cellular abnormalities seen in HGPS, the mechanism linking progerin to the organismal phenotype is not fully understood. To begin to address the mechanism behind HGPS using Drosophila melanogaster, we have ectopically expressed progerin and lamin A. We found that ectopic progerin and lamin A phenocopy several effects of laminopathies in developing and adult Drosophila, but that progerin causes a stronger phenotype than wild-type lamin A.
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