Biology:Sticky mouse
Sticky mouse is a murine possessing a gene mutation in the enzyme alanyl-tRNA synthetase (AARS).[1] The sticky mouse, with this particular mutation, presents a good model in which to investigate mechanisms of neuronal degeneration. Its most immediately obvious symptom is a sticky secretion on the mouse's fur (thus the name); however, it is accompanied by lack of muscle control, ataxia, alopecia, loss of Purkinje cells in the cerebellum, and eventually, death.
Sticky mouse is one of several animal mutants that are known to have problems in mRNA translation and are used in studies.[2].
A genetic mutation affecting the laminin α2 chain has been characterized in mouse models generated through targeted disruption of the Lama2 gene. These mutant mice exhibit severe muscular dystrophy, growth retardation, and early postnatal death. The absence of laminin α2 leads to progressive muscle fiber degeneration associated with increased apoptotic activity.[3]
See also
- Wasted mouse (wst) - EEF1A2 defect
- Harlequin mouse
- Reeler - RELN defect
- Shaking rat Kawasaki - RELN defect
References
- ↑ "Molecular biology: sticky end in protein synthesis". Nature 443 (7107): 41–2. September 2006. doi:10.1038/nature05002. PMID 16906138. Bibcode: 2006Natur.443...41R.
- ↑ "Translation matters: protein synthesis defects in inherited disease". Nat. Rev. Genet. 8 (9): 711–23. September 2007. doi:10.1038/nrg2142. PMID 17680008.
- ↑ "Laminin alpha2 chain-null mutant mice by targeted disruption of the Lama2 gene: a new model of merosin (laminin 2)-deficient congenital muscular dystrophy". FEBS Letters 415 (1): 33–39. September 22, 1997. doi:10.1016/S0014-5793(97)01007-7. PMID 9326364.
External links
- A sticky wicket - editor's summary, Nature, 2006
- Forward Genetics Reveals Novel Mechanisms of Neurodegeneration - free online videolecture by Ackerman SL.; June 2009
- 'Sticky' mice lead to discovery of new cause of neurodegenerative disease - Howard Hughes Medical Institute, 2006
- Susan L. Ackerman - bio at the Institute site
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