Description

Strain Information

Type Mutant Stock; Additional information on Genetically Engineered Mutant Mice. Mating System TJL Breeding Summary: homozygote x untyped sibling (+/?) then heterozygote x heterozygote. Species laboratory mouse Generation N1F20 (23-JAN-08)

Appearance
agouti, ataxic
Related Genotype: A/A Dab1^scm/Dab1^scm

agouti, unaffected
Related Genotype: A/A Dab1^scm/Dab1^scm or A/A +/?

Description
Mice homozygous for the scrambler spontaneous mutation (Dab1^scm) are recognized by an unstable gait and whole-body tremor. The cerebella of 30-day-old scrambler homozygotes are hypoplastic and devoid of folia; however, neither seizures nor abnormal brain wave patterns have been observed. Scrambler is similar to the reeler mutation in phenotype and pathology and, like reeler, probably results from defective neuronal migration. Female homozygotes mate and breed. Homozygous scrambler mutants have an ataxic gait which in the male may be contributory factor in the failure to mate. Normal life span for both sexes.

Control Information

	Control
	Untyped from the colony
Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Dab1

003581	CBy.129S4-Dab1tm1Cpr/J
003925	MRL.129P2(B6)-B2mtm1Unc/Dcr-Dab1scm-2J/J

View Strains carrying other alleles of Dab1     (2 strains)

Additional Web Information

JAX^® NOTES, Summer 2007; 506. JAX^® In Vivo Services Drug Efficacy Studies: Sample Morris Water Maze Study.

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms

      assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX^® Mice strain.

Dab1^scm/Dab1^scm

        involves: C3H * C57BL/6J

• nervous system phenotype
• abnormal retinal rod bipolar cell morphology (MGI Ref ID J:71970)
  • density of cells is decreased, indicating a decreased number of rod bipolar cells compared to controls
• vision/eye phenotype
• abnormal cone electrophysiology (MGI Ref ID J:71970)
  • cone mediated-responses are reduced in amplitude and delayed in mutants; difference compared to wild-type cone function is not as great as differences in rod function
• abnormal retinal inner nuclear layer morphology (MGI Ref ID J:71970)
  • density of synaptic terminals is decreased compared to controls
• abnormal retinal rod bipolar cell morphology (MGI Ref ID J:71970)
  • density of cells is decreased, indicating a decreased number of rod bipolar cells compared to controls
• abnormal rod electrophysiology (MGI Ref ID J:71970)
  • amplitude of a-wave of ERG in response to bright light flash is smaller than in controls while the b-wave is significantly reduced
  • oscillatory potentials (OPs) are reduced in amplitude

View Research Applications

Research Applications

This mouse can be used to support research in many areas including:

Dab1^scm related

Developmental Biology Research
Neurodevelopmental Defects

Neurobiology Research
Ataxia (Movement) Defects
Cerebellar Defects
Cortical Defects
Neurodevelopmental Defects
Tremor Defects

Genes & Alleles

Gene & Allele Information

Allele Symbol		Dab1scm
Allele Name		scrambler
Allele Type		Spontaneous
Common Name(s)		scm;
Strain of Origin		DC/LeJ
Gene Symbol and Name		Dab1, disabled homolog 1 (Drosophila)
Chromosome		4
Gene Common Name(s)		AI956902; C630028C02Rik; RIKEN cDNA C630028C02 gene; expressed sequence AI956902; scm; scr; scrambler; yot; yotari;
Molecular Note		Transcripts produced from this allele show abnormal splicing and are predicted to produce a truncated protein. Sequence analysis revealed that the larger scrambler transcript contains 1.5 kb of intracisternal A particle retrotransposon (IAP) sequence inantisense orientation. Previous mapping studies detcted an IAP particle that failed to recombine with the Dab1 locus. Thus, it appears that abberrant splicing has incorporated the IAP element in the coding region of the gene. [MGI Ref ID J:42628] [MGI Ref ID J:43504]

Genotyping

Genotyping Information

This strain will not have a genotyping protocol or one is not currently available.

Helpful Links

Optimizing PCR Protocols

References

References

Additional References

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. [PubMed: 8875886]  [MGI Ref ID J:37166]

Ware ML; Fox JW; Gonzalez JL; Davis NM; Lambert de Rouvroit C ; Russo CJ ; Chua SC Jr ; Goffinet AM ; Walsh CA. 1997. Aberrant splicing of a mouse disabled homolog, mdab1, in the scrambler mouse. Neuron 19(2):239-49. [PubMed: 9292716]  [MGI Ref ID J:42628]

Weiss KH; Johanssen C; Tielsch A; Herz J; Deller T; Frotscher M; Forster E. 2003. Malformation of the radial glial scaffold in the dentate gyrus of reeler mice, scrambler mice, and ApoER2/VLDLR-deficient mice. J Comp Neurol 460(1):56-65. [PubMed: 12687696]  [MGI Ref ID J:83151]

Dab1^scm related

Akopians AL; Babayan AH; Beffert U; Herz J; Basbaum AI; Phelps PE. 2008. Contribution of the Reelin signaling pathways to nociceptive processing. Eur J Neurosci 27(3):523-37. [PubMed: 18279306]  [MGI Ref ID J:132269]

Borrell V; Pujadas L; Simo S; Dura D; Sole M; Cooper JA; Del Rio JA; Soriano E. 2007. Reelin and mDab1 regulate the development of hippocampal connections. Mol Cell Neurosci 36(2):158-73. [PubMed: 17720534]  [MGI Ref ID J:126745]

Cariboni A; Rakic S; Liapi A; Maggi R; Goffinet A; Parnavelas JG. 2005. Reelin provides an inhibitory signal in the migration of gonadotropin-releasing hormone neurons. Development 132(21):4709-18. [PubMed: 16207762]  [MGI Ref ID J:102848]

Chung S; Zhang Y; Van Der Hoorn F; Hawkes R. 2007. The anatomy of the cerebellar nuclei in the normal and scrambler mouse as revealed by the expression of the microtubule-associated protein kinesin light chain 3. Brain Res 1140:120-31. [PubMed: 17447264]  [MGI Ref ID J:121113]

David A; Tiveron MC; Defays A; Beclin C; Camosseto V; Gatti E; Cremer H; Pierre P. 2007. BAD-LAMP defines a subset of early endocytic organelles in subpopulations of cortical projection neurons. J Cell Sci 120(Pt 2):353-65. [PubMed: 17215451]  [MGI Ref ID J:117449]

Goldowitz D; Cushing RC; Laywell E; D'Arcangelo G; Sheldon M; Sweet HO; Davisson M; Steindler D; Curran T. 1997. Cerebellar disorganization characteristic of reeler in scrambler mutant mice despite presence of reelin. J Neurosci 17(22):8767-77. [PubMed: 9348346]  [MGI Ref ID J:44084]

Gonzalez 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. [PubMed: 9364067]  [MGI Ref ID J:44289]

Gupta A; Sanada K; Miyamoto DT; Rovelstad S; Nadarajah B; Pearlman AL; Brunstrom J; Tsai LH. 2003. Layering defect in p35 deficiency is linked to improper neuronal-glial interaction in radial migration. Nat Neurosci 6(12):1284-91. [PubMed: 14608361]  [MGI Ref ID J:86637]

Jossin Y; Goffinet AM. 2007. Reelin signals through phosphatidylinositol 3-kinase and Akt to control cortical development and through mTor to regulate dendritic growth. Mol Cell Biol 27(20):7113-24. [PubMed: 17698586]  [MGI Ref ID J:126769]

Jossin Y; Gui L; Goffinet AM. 2007. Processing of Reelin by embryonic neurons is important for function in tissue but not in dissociated cultured neurons. J Neurosci 27(16):4243-52. [PubMed: 17442808]  [MGI Ref ID J:121108]

Rice DS; Nusinowitz S; Azimi AM; Martinez A; Soriano E; Curran T. 2001. The reelin pathway modulates the structure and function of retinal synaptic circuitry. Neuron 31(6):929-41. [PubMed: 11580894]  [MGI Ref ID J:71970]

Rice DS; Sheldon M; D'Arcangelo G; Nakajima K; Goldowitz D; Curran T. 1998. Disabled-1 acts downstream of Reelin in a signaling pathway that controls laminar organization in the mammalian brain. Development 125(18):3719-29. [PubMed: 9716537]  [MGI Ref ID J:50400]

Rossel M; Loulier K; Feuillet C; Alonso S; Carroll P. 2005. Reelin signaling is necessary for a specific step in the migration of hindbrain efferent neurons. Development 132(6):1175-85. [PubMed: 15703280]  [MGI Ref ID J:97218]

Sanada K; Gupta A; Tsai LH. 2004. Disabled-1-regulated adhesion of migrating neurons to radial glial fiber contributes to neuronal positioning during early corticogenesis. Neuron 42(2):197-211. [PubMed: 15091337]  [MGI Ref ID J:90026]

Sheldon M; Rice DS; D'Arcangelo G; Yoneshima H; Nakajima K; Mikoshiba K ; Howell BW ; Cooper JA ; Goldowitz D ; Curran T. 1997. Scrambler and yotari disrupt the disabled gene and produce a reeler-like phenotype in mice [see comments] Nature 389(6652):730-3. [PubMed: 9338784]  [MGI Ref ID J:43504]

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. [PubMed: 8875886]  [MGI Ref ID J:37166]

Ware ML; Fox JW; Gonzalez JL; Davis NM; Lambert de Rouvroit C ; Russo CJ ; Chua SC Jr ; Goffinet AM ; Walsh CA. 1997. Aberrant splicing of a mouse disabled homolog, mdab1, in the scrambler mouse. Neuron 19(2):239-49. [PubMed: 9292716]  [MGI Ref ID J:42628]

Weiss KH; Johanssen C; Tielsch A; Herz J; Deller T; Frotscher M; Forster E. 2003. Malformation of the radial glial scaffold in the dentate gyrus of reeler mice, scrambler mice, and ApoER2/VLDLR-deficient mice. J Comp Neurol 460(1):56-65. [PubMed: 12687696]  [MGI Ref ID J:83151]

Yang H; Jensen P; Goldowitz D. 2002. The community effect and Purkinje cell migration in the cerebellar cortex: analysis of scrambler chimeric mice. J Neurosci 22(2):464-70. [PubMed: 11784791]  [MGI Ref ID J:73748]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           FGB29

Colony Maintenance

Mating System	TJL Breeding Summary: homozygote x untyped sibling (+/?) then heterozygote x heterozygote.
Diet Information	LabDiet® 5K52/5K67

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Supply Details

Standard Supply

Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.

Supply Notes

Usually shipped between four and eight weeks of age. This strain is included in the Mouse Mutant Resource collection. Genomic DNA is available for this strain from the Mouse DNA Resource.

Control Information

	Control
	Untyped from the colony
Considerations for Choosing Controls
USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
International - Control Pricing Information for Genetically Engineered Mutant Strains.

General Terms and Conditions

See Terms of Use

The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX^® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX^® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX^® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.

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Ordering and Purchasing Information

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Contact Information
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Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form

Terms of Use

Terms of Use

General Terms and Conditions

Contact information

General inquiries

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phone:	207-288-6470
fax:	207-288-6655

JAX^® Mice & Services Conditions of Use

“Each recipient institution, including its employees and other researchers under its control (RECIPIENT), of mice or services using mice from The Jackson Laboratory (TJL) agrees that such mice, descendants of those mice derived by inbreeding or crossbreeding, including unmodified derivatives of those mice or their descendants (“MICE”) shall not be: (i) used for any purpose other than the internal research of the RECIPIENT, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services with respect to MICE. Acceptance of MICE from TJL shall be deemed agreement by RECIPIENT to these conditions, and departure from these conditions requires The Jackson Laboratory’s prior written authorization.”

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of MICE, products or services, The Jackson Laboratory will, at its option, provide credit or replacement for the MICE or product received or the services provided.

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