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Disruption of SMN function by ectopic expression of the human SMN gene in Drosophila.

Abstract:

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 anal...

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Publication status:
Published

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Institution:
University of Oxford
Division:
MSD
Department:
Physiology Anatomy & Genetics
Role:
Author
Journal:
FEBS letters More from this journal
Volume:
486
Issue:
2
Pages:
99-102
Publication date:
2000-12-01
DOI:
EISSN:
1873-3468
ISSN:
0014-5793

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