B6EiC3Sn-Rb(12.Ts17¹⁶65Dn)2Cje/CjeDnJ

Stock number: 004850
Product name: Ts[Rb(12.17(16))]2Cje
Research areas: Mouse/Human Gene Homologs, Neurobiology Research, Research Tools

Description

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.

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.

Allele

Symbol: Rb(12.Ts17¹⁶65Dn)2Cje
Name: Robertsonian translocation, Chr 12 and T17<16>Dn, Charles J Epstein 2
MGI accession ID: MGI:3531161
Generation method: Not Applicable
Marker symbol: Rb(12.Ts17¹⁶65Dn)2Cje
Marker name: Robertsonian translocation, Chr 12 and T17<16>Dn, Charles J Epstein 2
Chromosome: UN
Strain of origin: Not Specified
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.