Scrambler mouse: Difference between revisions
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'''Scrambler''' is a mutant mouse lacking functional [[DAB1]] gene, resulting in [[reeler]]-like phenotype.<!-- |
'''Scrambler''' is a mutant mouse lacking a functional [[DAB1]] gene, resulting in [[reeler]]-like phenotype.<!-- |
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--><ref name="pmid9364067">{{cite journal |author=González JL, Russo CJ, Goldowitz D, Sweet HO, Davisson MT, Walsh CA |title=Birthdate and cell marker analysis of scrambler: a novel mutation affecting cortical development with a reeler-like phenotype |journal=[[J. Neurosci.]] |volume=17 |issue=23 |pages=9204–11 |year=1997 |month=December |pmid=9364067 |doi= |url=http://www.jneurosci.org/cgi/pmidlookup?view=long&pmid=9364067}}</ref><!-- |
--><ref name="pmid9364067">{{cite journal |author=González JL, Russo CJ, Goldowitz D, Sweet HO, Davisson MT, Walsh CA |title=Birthdate and cell marker analysis of scrambler: a novel mutation affecting cortical development with a reeler-like phenotype |journal=[[J. Neurosci.]] |volume=17 |issue=23 |pages=9204–11 |year=1997 |month=December |pmid=9364067 |doi= |url=http://www.jneurosci.org/cgi/pmidlookup?view=long&pmid=9364067}}</ref><!-- |
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--><ref name="pmid8875886">{{cite journal |author=Sweet HO, Bronson RT, Johnson KR, Cook SA, Davisson MT |title=Scrambler, a new neurological mutation of the mouse with abnormalities of neuronal migration |journal=[[Mamm. Genome]] |volume=7 |issue=11 |pages=798–802 |year=1996 |month=November |pmid=8875886 |doi= 10.1007/s003359900240|url=http://link.springer-ny.com/link/service/journals/00335/bibs/7n11p798.html}}</ref> |
--><ref name="pmid8875886">{{cite journal |author=Sweet HO, Bronson RT, Johnson KR, Cook SA, Davisson MT |title=Scrambler, a new neurological mutation of the mouse with abnormalities of neuronal migration |journal=[[Mamm. Genome]] |volume=7 |issue=11 |pages=798–802 |year=1996 |month=November |pmid=8875886 |doi= 10.1007/s003359900240|url=http://link.springer-ny.com/link/service/journals/00335/bibs/7n11p798.html}}</ref> |
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The spontaneous [[autosomal]] [[recessive]] scrambler mutation on [[chromosome 4]] causes a deficiency of Disabled-1, involved in Reelin signaling (Rice et al 1998; Sweet et al 1996). Dba1-scm homozygous mutants possess a reeler-like phenotype with respect to cell malpositioning in cerebellar cortex, hippocampus, and neocortex (Gonzalez et al 1997; Rice et al 1998; Sheldon et al 1997; Sweet et al 1996; Weiss et al 2003), but intact deep cerebellar nuclei (Chung et al 2007). Analysis of Dba1-scm [[chimeras]] indicates that mutant [[Purkinje cells]] are not aided by wild-type cells (Yang et al 2002). Purkinje and granule cell degeneration results in [[ataxia]] (Goldowitz et al 1997). Despite normal Reln mRNA levels, Dba1-scm mutants have defective reelin signaling, indicating that disabled-1 acts downstream of reelin. Cell ectopias are identical with targeted disruption of Dba1 (Gallagher et al 1998; Howell et al 1997). |
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==References== |
==References== |
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Revision as of 12:52, 2 March 2011
Scrambler is a mutant mouse lacking a functional DAB1 gene, resulting in reeler-like phenotype.[1] The strain was first described in 1996.[2]
The spontaneous autosomal recessive scrambler mutation on chromosome 4 causes a deficiency of Disabled-1, involved in Reelin signaling (Rice et al 1998; Sweet et al 1996). Dba1-scm homozygous mutants possess a reeler-like phenotype with respect to cell malpositioning in cerebellar cortex, hippocampus, and neocortex (Gonzalez et al 1997; Rice et al 1998; Sheldon et al 1997; Sweet et al 1996; Weiss et al 2003), but intact deep cerebellar nuclei (Chung et al 2007). Analysis of Dba1-scm chimeras indicates that mutant Purkinje cells are not aided by wild-type cells (Yang et al 2002). Purkinje and granule cell degeneration results in ataxia (Goldowitz et al 1997). Despite normal Reln mRNA levels, Dba1-scm mutants have defective reelin signaling, indicating that disabled-1 acts downstream of reelin. Cell ectopias are identical with targeted disruption of Dba1 (Gallagher et al 1998; Howell et al 1997).
References
- ^ González JL, Russo CJ, Goldowitz D, Sweet HO, Davisson MT, Walsh CA (1997). "Birthdate and cell marker analysis of scrambler: a novel mutation affecting cortical development with a reeler-like phenotype". J. Neurosci. 17 (23): 9204–11. PMID 9364067.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Sweet HO, Bronson RT, Johnson KR, Cook SA, Davisson MT (1996). "Scrambler, a new neurological mutation of the mouse with abnormalities of neuronal migration". Mamm. Genome. 7 (11): 798–802. doi:10.1007/s003359900240. PMID 8875886.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link)