Beard Gemma S, Bridger Joanna M, Kill Ian R, Tree David R P
Division of Biosciences, Centre for Cell and Chromosome Biology, School of Health Sciences and Social Care, Brunel University, Uxbridge, Middlesex, UK.
Biochem Soc Trans. 2008 Dec;36(Pt 6):1389-92. doi: 10.1042/BST0361389.
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.
层粘连蛋白病哈钦森-吉尔福德早衰综合征（HGPS）由突变的核纤层蛋白A（progerin）引起，导致患病儿童过早衰老。尽管对HGPS中细胞异常的基础有了许多细胞生物学和生物化学方面的见解，但将progerin与机体表型联系起来的机制仍未完全了解。为了开始利用黑腹果蝇研究HGPS背后的机制，我们异位表达了progerin和核纤层蛋白A。我们发现，异位表达的progerin和核纤层蛋白A模拟了层粘连蛋白病在发育中和成年果蝇中的几种效应，但progerin比野生型核纤层蛋白A导致更强的表型。
