title: Towards a Drosophila model of Hutchinson-Gilford progeria syndrome.
abstract: 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.
authors.affiliation: Division of Biosciences, Centre for Cell and Chromosome Biology, School of Health Sciences and Social Care, Brunel University, Uxbridge, Middlesex, UK.
authors.forename: Gemma S
authors.initials: GS
authors.lastname: Beard
authors.forename: Joanna M
authors.initials: JM
authors.lastname: Bridger
authors.forename: Ian R
authors.initials: IR
authors.lastname: Kill
authors.forename: David R P
authors.initials: DR
authors.lastname: Tree
mesh_terms: D000818:Animals; D002467:Cell Nucleus / Q000378:metabolism / Q000473:pathology; D004195:Disease Models, Animal; D004331:Drosophila melanogaster / Q000196:embryology / Q000378:metabolism*; D034904:Lamin Type A / Q000378:metabolism; D008136:Longevity; D011371:Progeria / Q000473:pathology*
chemical_list: D034904:Lamin Type A