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| Scrambler mouse ： ウィキペディア英語版 |
Scrambler mouse
Scrambler is a spontaneous mouse mutant lacking a functional DAB1 gene, resulting in a phenotype resembling that seen in the reeler mouse.〔González JL, Russo CJ, Goldowitz D, Sweet HO, Davisson MT, Walsh CA. Birthdate and cell marker analysis of scrambler: a novel mutation affecting cortical development with a reeler-like phenotype. ''J Neurosci'' 17, 9204–11, 1997, PMID 9364067〕 The strain was first described by Sweet ''et al.'' in 1996.〔Sweet HO, Bronson RT, Johnson KR, Cook SA, Davisson MT |title=Scrambler, a new neurological mutation of the mouse with abnormalities of neuronal migration. ''Mamm Genome'' 7, 798–802, 1996, PMID 8875886〕
== Neuroanatomical abnormalities ==
The spontaneous autosomal recessive scrambler mutation on chromosome 4 causes a deficiency of DAB1, encoding disabled-1, a protein involved in the signaling of the Reelin protein, lacking in the reeler mutant,〔Rice DS, Sheldon M, D'Arcangelo G, Nakajima K, Goldowitz D, Curran T. Disabled-1 acts downstream of Reelin in a signaling pathway that controls laminar organization in the mammalian brain. ''Development'' 125, 3719-29, 1998, PMID 9716537〕 Dab1-scm homozygous mutants possess a reeler-like phenotype with respect to cell malpositioning in cerebellar cortex, hippocampus, and neocortex.〔Sheldon M, Rice DS, D'Arcangelo G, Yoneshima H, Nakajima K, Mikoshiba K, Howell BW, Cooper JA, Goldowitz D, Curran T. Scrambler and yotari disrupt the disabled gene and produce a reeler-like phenotype in mice. ''Nature'' 389, 730-3, 1997, PMID 11784791〕〔Weiss KH, Johanssen C, Tielsch A, Herz J, Deller T, Frotscher M, Förster E. Malformation of the radial glial scaffold in the dentate gyrus of reeler mice, scrambler mice, and ApoER2/VLDLR-deficient mice. ''J Comp Neurol'' 460, 56-65, 2003, PMID 12687696.〕 Purkinje cell and granule cell degeneration results in ataxia.〔Goldowitz D, Cushing RC, Laywell E, D'Arcangelo G, Sheldon M, Sweet HO, Davisson M, Steindler D, Curran T. Cerebellar disorganization characteristic of reeler in scrambler mutant mice despite presence of reelin. ''J Neurosci'' 17: 8767-77, 1997, PMID 9348346〕 Despite normal Reln mRNA levels, Dab1-scm mutants have defective reelin signaling, indicating that disabled-1 acts downstream of reelin. Cell ectopias are identical with targeted disruption of Dab1.〔Gallagher E, Howell BW, Soriano P, Cooper JA, Hawkes R. Cerebellar abnormalities in the disabled (mdab1-1) mouse. ''J Comp Neurol'' 402, 238-51, 1998, PMID 9845246〕〔Howell BW, Hawkes R, Soriano P, Cooper JA. Neuronal position in the developing brain is regulated by mouse disabled-1. ''Nature'' 389, 733-7, 1997, PMID 9338785〕
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