期刊
FEBS LETTERS
卷 486, 期 2, 页码 99-102出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/S0014-5793(00)02243-2
关键词
motor neuron disease; spinal muscular atrophy; survival motor neuron; transgenic; Drosophila
Spinal muscular atrophy is a neurodegenerative disorder caused by mutations or deletions in the survival motor neuron (SMN) gene. We have cloned the Drosophila ortholog of SMN (DmSMN) and disrupted its function by ectopically expressing human SMN. This leads to pupal lethality caused by a dominant-negative effect, whereby human SMN may bind endogenous DmSMN resulting in non-functional DmSMN/human SMN hetero-complexes. Ectopic expression of truncated versions of DmSMN and yeast two-hybrid analysis show that the C-terminus of SMN is necessary and sufficient to replicate this effect. We have therefore generated a system which can be utilized to carry out suppressor and high-throughput screens, and provided in vivo evidence for the importance of SMN oligomerization for SMN function at the level of an organism as a whole. (C) 2000 Federation of European Biochemical Societies. Published by Elsevier Science B.V. All rights reserved.
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