Overview

This strain carries the same trisomic segment as other strains that are models for Down syndrome, but may have advantages in that transmission of the trisomic segment through the female germline is greater and males are fertile.

Genetic overview

Genetic Background	Generation
	N11+N28 (2021-01-06 00:00:00)

Rb(12.Ts17¹⁶65Dn)2Cje

Allele Type	Gene Symbol	Gene Name
Not Applicable	Rb(12.Ts17¹⁶65Dn)2Cje	Robertsonian translocation, Chr 12 and T17<16>Dn, Charles J Epstein 2

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Research Applications

Neurobiology Research
Mouse/Human Gene Homologs

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Base Price

Starting at:

$247.50 Domestic price for female 4-week

408.00 Domestic price for breeder pair

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Details

Important Note

Pde6b^rd1, the recessive retinal degeneration 1 mutation, is segregating in this colony. Animals that are homozygous for rd1 will be blind.

Detailed Description

The trisomic segment in the Rb(12.Ts17¹⁶65Dn)2Cje strain is genetically identical to the Ts65Dn strains, Stock No. 001924 and Stock No. 005252, with trisomy of the Chr 16 genes, from Mrpl39 to the distal telomere. Quantitative PCR confirmed the triplication of Chr 16 genes from App to Mx1. Transmission of the chromosome 16 segmental trisomy through the female germline is significantly improved over Ts65Dn (43% versus 24%). Dendritic spines on granule cells in the fascia dentata are enlarged in size and decreased in density (Villar AJ, et al. 2005). Unlike Ts65Dn, males are fertile. Trisomic mice are 20% smaller in size than controls. This strain serves a model for Down syndrome.

The Clcc1^m1J spontaneous mutation, which causes increased sensitivity to endoplasmic reticulum stress in the cerebellum, is a homozygous strain characteristic of C3H/HeSnJ (Jia et al., 2015) so is segregating in this strain.

Development

The Rb(12.Ts171665Dn)2Cje strain was the result of a spontaneous fusion between the small Ts65Dn marker chromosome (carrying the distal end of Chr 16) and Chr 12, forming a Robertsonian fusion chromosome. This Robertsonian chromosome was found among the progeny of a mating between a trisomic Ts65Dn female, B6EiC3Sn a/A - Ts(17¹⁶)65Dn (Stock No. 001924) and a diploid B6EiC3SnF1 male. Quantitative PCR confirmed the triplication of Chr 16 genes from App to Mx1, the same as in Ts65Dn strains.

Control Suggestions

Wild-type from the colony

Approximate Controls

001875 B6EiC3SnF1/J

Additional Information

Considerations for Choosing Controls

Selected References

Villar AJ; Belichenko PV; Gillespie AM; Kozy HM; Mobley WC; Epstein CJ. 2005. Identification and characterization of a new Down syndrome model, Ts[Rb(12.1716)]2Cje, resulting from a spontaneous Robertsonian fusion between T(171)65Dn and mouse chromosome 12. Mamm Genome 16(2):79-90PubMed: 15859352 MGI: J:96650

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Genetics

Rb(12.Ts17¹⁶65Dn)2Cje

Allele Symbol: Rb(12.Ts17¹⁶65Dn)2Cje

Allele Name	Robertsonian translocation, Chr 12 and T17<16>Dn, Charles J Epstein 2
Allele Type	Not Applicable
Allele Synonym(s)	Rb2Cje; Ts(Rb(12.17¹⁶))2Cje; Ts2; Ts2Cje
Gene Symbol and Name	Rb(12.Ts17¹⁶65Dn)2Cje, Robertsonian translocation, Chr 12 and T17<16>Dn, Charles J Epstein 2
Gene Synonym(s)
Strain of Origin	Not Specified
Chromosome	UN
Molecular Note	A female mouse with 40 chromosomes and 41 arms was detected by chromosome analysis of progeny from T17¹⁶65Dn mice. This mouse had a unique biarmed chromosome that appeared to be the result of a fusion between chromosome 12 and the marker chromosome T(17¹⁶)65Dn.

Disease/Phenotype

Disease Terms

Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.

Down syndrome

Research Areas By Phenotype

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

Neurobiology Research
- Down syndrome
Mouse/Human Gene Homologs
- Down syndrome

Mammalian Phenotype Terms by Genotype

The following phenotype information is associated with a similar, but not exact match to this JAX^® Mice strain

Genotype: Rb(12.Ts17¹⁶65Dn)2Cje/0
Not Specified

nervous system phenotype

abnormal nervous system electrophysiology
- long-term potentiation induced by tetanic stimulation (100 Hz, 1 s) in the CA1 region of hippocampal slices is mostly intact, however depolarization during high-frequency stimulation (depolarization envelope) 950 ms after the first pulse of tetanic stimulation is smaller

The following phenotype relates to a compound genotype created using this strain

Genotype: Rb(12.Ts17¹⁶65Dn)2Cje/0
involves: C3H/HeSnJ * C57BL/6JEi

cellular phenotype

increased neuron apoptosis
- the number of apoptotic neurons in the cortex is more than double that of controls

reproductive system phenotype

decreased testis weight
- testes are 65.6% of control testes weights

endocrine/exocrine gland phenotype

decreased testis weight
- testes are 65.6% of control testes weights

growth/size/body region phenotype

decreased body size
- mice are ~20% smaller in size compared to normal littermates postnatally, which persists throughout life

mammalian phenotype

reproductive system phenotype
- mice are viable and fertile
- testes are structurally indistinguishable from controls and have normal progression of spermatogenesis

nervous system phenotype

abnormal neuron morphology
- spine density in dentate granule cells is decreased (by ~15%) compared to controls; decrease is significant for inner and middle third portions of the molecular layer of the fascia dentate
- dendritic spines are significantly enlarged, with ~13% having a head area larger than 0.5 um² compared to 2% in controls; spines have significantly shorter necks, by ~30% compared to controls

abnormal hippocampus morphology
- basal calcium levels of hippocampal neurons in culture is elevated compared to controls

increased neuron apoptosis
- the number of apoptotic neurons in the cortex is more than double that of controls

loss of hippocampal neurons
- 50% of hippocampal neurons cultured in vitro die after 7 days compared to just 15% for controls

References

Villar AJ; Belichenko PV; Gillespie AM; Kozy HM; Mobley WC; Epstein CJ. 2005. Identification and characterization of a new Down syndrome model, Ts[Rb(12.1716)]2Cje, resulting from a spontaneous Robertsonian fusion between T(171)65Dn and mouse chromosome 12. Mamm Genome 16(2):79-90PubMed: 15859352 MGI: J:96650

Additional - Rb(12.Ts17¹⁶65Dn)2Cje related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- QPCR:Trisomy QPCR
- Separated PCR:Clcc1
- QPCR:Trisomy QPCR
- Standard PCR:Generic Pde6b
- Separated PCR:Ts(17<16>)
- Standard PCR:Ts17<16>
- Standard PCR:Ts17<16>
- Standard PCR:Generic Pde6b Alternate1
- Probe:Pde6b Probe
- Genotyping resources and troubleshooting
Dietary Information
- LabDiet® 5K52 formulation (6% fat)
Mating System
- Rb(12.Ts17¹⁶65Dn)2Cje female x B6EiC3SnF1/J (001875) male or reciproca
Appearance
- agouti or black
  Related Genotype: A/A or A/a or a/a
Citation
When using the B6EiC3Sn-Rb(12.Ts17¹⁶65Dn)2Cje/CjeDnJ mouse strain in a publication, please cite the originating article(s) and include JAX stock #004850 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

AX11 (Maximum)

Pricing & Availability

Available

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Live Mouse

Age

Genotype

Price

weeks

Breeder Pair

Sex

Genotype

Price

Female
Male

Cryorecovery - Pricing

Service/Product	Description	Price
> >	Heterozygous Females and Wild-type Males for Rb(12.Ts17<16>65Dn)2Cje

Related Products and Services

Frozen Mouse Embryo	B6EiC3Sn-Rb(12.Ts17<16>65Dn)2Cje/CjeDnJ Frozen Embryos	$2595.00
Frozen Mouse Embryo	B6EiC3Sn-Rb(12.Ts17<16>65Dn)2Cje/CjeDnJ Frozen Embryos	$2595.00
Frozen Mouse Embryo	B6EiC3Sn-Rb(12.Ts17<16>65Dn)2Cje/CjeDnJ Frozen Embryos	$3373.50
Frozen Mouse Embryo	B6EiC3Sn-Rb(12.Ts17<16>65Dn)2Cje/CjeDnJ Frozen Embryos	$3373.50

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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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

Terms Of Use

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Additional Use Restrictions Apply

Strain from the Cytogenetic Models Resource. Each order requires submission of a Request Form, please inquire.

Licensing Information

Phone: 207-288-6470

Email: TechTran@jax.org

Genetic overview

Research Applications

Base Price

Important Note

Detailed Description

Development

Control Suggestions

Approximate Controls

Additional Information

Selected References

Rb(12.Ts171665Dn)2Cje

Allele Symbol: Rb(12.Ts171665Dn)2Cje

Disease Terms

Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.

Research Areas By Phenotype

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

Mammalian Phenotype Terms by Genotype

Genotype: Rb(12.Ts171665Dn)2Cje/0Not Specified

nervous system phenotype

Genotype: Rb(12.Ts171665Dn)2Cje/0involves: C3H/HeSnJ * C57BL/6JEi

cellular phenotype

reproductive system phenotype

endocrine/exocrine gland phenotype

growth/size/body region phenotype

mammalian phenotype

nervous system phenotype

References

Additional - Rb(12.Ts171665Dn)2Cje related

Genotyping Protocols

Dietary Information

Mating System

Appearance

Citation

Animal Health Reports

Live Mouse

Breeder Pair

Cryorecovery - Pricing

Related Products and Services

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms Of Use

Additional Use Restrictions Apply

Licensing Information

Rb(12.Ts17¹⁶65Dn)2Cje

Allele Symbol: Rb(12.Ts17¹⁶65Dn)2Cje

Genotype: Rb(12.Ts17¹⁶65Dn)2Cje/0
Not Specified

Genotype: Rb(12.Ts17¹⁶65Dn)2Cje/0
involves: C3H/HeSnJ * C57BL/6JEi

Additional - Rb(12.Ts17¹⁶65Dn)2Cje related