Description

Strain Information

Former Names B6;129S7-Dp(17Abcg1-Rrp1b)1Yey/J    (Changed: 17-AUG-11 ) Type Duplication; Additional information on Mice with Chromosomal Aberrations. Type Congenic; Mutant Stock; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Mating System Noncarrier x Hemizygote         (Female x Male)   20-SEP-12 Mating System Hemizygote x Noncarrier         (Female x Male)   20-SEP-12 Species laboratory mouse   Donating Investigator Y. Eugene Yu,   Roswell Park Cancer Institute

Description
This Dp(17)3Yey/+ mutant strain contains one copy of mouse Chromosome 17 with the targeted sequence duplicated between, and including, the ATP-binding cassette, sub-family G (WHITE), member 1 (Abcg1) gene and ribosomal RNA processing 1 homolog B (S. cerevisiae) (Rrp1b) gene. Hemizygous mice are viable and fertile. This duplicated region on the mouse chromosome is one of three regions orthologous to an extra copy of human Chromosome 21 (Hsa21) which has been implicated in developmental cognitive disabilities associated with Down Syndrome (DS). Dp(17)1Yey/+ contains a duplication syntenic to the proximal part of human 21q22.3, and carries 19 genes orthologous to genes on Hsa21. When mice carrying this transgene are bred to B6;129-Dp(10Prmt2-Pdxk)2Yey/J mice (Stock No. 013529) and B6;129-Dp(16Lipi-Zfp295)1Yey/J mice (Stock No. 013530) to create a triple transgenic strain containing all Hsa21 regions, the resulting mice exhibit DS related neurological defects. These mice display reduction in body length and weight, hydrocephaly caused by aqueductal stenosis, impaired grip strength, impaired learning, and a deficit in long-term potentiation. 26% of triple transgenic offspring die shortly after birth due to heart defects. These mice may be useful for understanding developmental and cognitive disabilities associated with DS.

Development
Chromosome-engineering cassettes were inserted into mouse Chromosome 17 of 129S7/SvEvBrd-Hprt1^b-m2-derived AB2.2 embryonic stem (ES) cells, bracketing a span of approximately 1.1 Mb between, and including, the ATP-binding cassette, sub-family G (WHITE), member 1 (Abcg1) gene and ribosomal RNA processing 1 homolog B (S. cerevisiae) (Rrp1b) loci. The cassette placed at the proximal locus was targeted upstream of Abcg1 and contained a tyrosinase minigene, a 5' portion of an HPRT minigene, a loxP site, and a neomycin resistance gene. The cassette placed at the distal locus was targeted downstream of the Rrp1b gene and contained a puromycin resistance gene, a loxP site, a 3' portion of an HPRT minigene, and an agouti transgene. Double-targeted ES cells were transiently transfected with the cre recombinase expression vector pOG231. After subsequent selection of recombinants by using hypoxanthine aminopterin thymidine (HAT) media, correctly targeted ES cells, containing one copy of Chromosome 17 with the targeted sequence duplicated (Dp), were injected into C57BL/6J-Tyr^c-Brd blastocysts. The resulting chimeric mice were mated to C57BL/6J mice and were subsequently backcrossed to C57BL/6J for at least 3 generations to produce a colony of Dp(17)3Yey/+ mice. Upon arrival at The Jackson Laboratory these mice were bred to C57BL/6J mice (Stock No. 000664) for at least one generation to establish the colony.

Control Information

	Control
	Noncarrier
	000664 C57BL/6J	(approximate)
Considerations for Choosing Controls

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI

      assigned by genotype

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

Dp(17Abcg1-Rrp1b)3Yey/0

        B6.129S7-Dp(17Abcg1-Rrp1b)3Yey

• mortality/aging
• *normal* mortality/aging
  • unlike mice carrying Dp(16Lipi -Zfp295)1Yey , no increase in neonatal lethality relative to wild-type controls is detected   (MGI Ref ID J:161325)

View Research Applications

Research Applications

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

Developmental Biology Research
Craniofacial and Palate Defects
      hydrocephaly: severely abnormal skull
Skeletal Defects
      hydrocephaly

Mouse/Human Gene Homologs
Down syndrome

Neurobiology Research
Behavioral and Learning Defects
Down syndrome

Genes & Alleles

Gene & Allele Information provided by MGI

Allele Symbol		Dp(17Abcg1-Rrp1b)3Yey
Allele Name		duplication, Chr 17, Y Eugene Yu 3
Allele Type		Targeted (knock-in)
Common Name(s)		Dp(17)1Yey; Dp(17Abcg1-Rrp1b)1Yey; Dp3Yey; Ts3Yey;
Strain of Origin		129S7/SvEvBrd-Hprt
Gene Symbol and Name		Dp(17Abcg1-Rrp1b)3Yey, duplication, Chr 17, Y Eugene Yu 3
Chromosome		17
Gene Common Name(s)		Dp(17)1Yey; Dp(17Abcg1-Rrp1b)1Yey; Ts3Yey; duplication, Chr 17, Y Eugene Yu 1;
Molecular Note		Cre mediated chromosomal rearrangement between loxP sites in regions proximal to Abcg1 and distal to Rrp1b results in duplication of the genes, including Abcg1 and Rrp1b, in this approximately 1.1 Mb region that is syntenic with the proximal part of human 21q22.3. [MGI Ref ID J:161325]

Genotyping

Genotyping Information

Genotyping Protocols

Dp(17Abcg1-Rrp1b)3Yey,

Separated MCA

Dp(17Abcg1-Rrp1b)3Yey, Separated PCR

Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Yu T; Li Z; Jia Z; Clapcote SJ; Liu C; Li S; Asrar S; Pao A; Chen R; Fan N; Carattini-Rivera S; Bechard AR; Spring S; Henkelman RM; Stoica G; Matsui S; Nowak NJ; Roder JC; Chen C; Bradley A; Yu YE. 2010. A mouse model of Down syndrome trisomic for all human chromosome 21 syntenic regions. Hum Mol Genet 19(14):2780-91. [PubMed: 20442137]  [MGI Ref ID J:161325]

Additional References

Dp(17Abcg1-Rrp1b)3Yey related

Yu T; Liu C; Belichenko P; Clapcote SJ; Li S; Pao A; Kleschevnikov A; Bechard AR; Asrar S; Chen R; Fan N; Zhou Z; Jia Z; Chen C; Roder JC; Liu B; Baldini A; Mobley WC; Yu YE. 2010. Effects of individual segmental trisomies of human chromosome 21 syntenic regions on hippocampal long-term potentiation and cognitive behaviors in mice. Brain Res 1366:162-71. [PubMed: 20932954]  [MGI Ref ID J:166931]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX18

Colony Maintenance

Breeding & Husbandry	When maintaining a live colony, hemizygous mice may be bred to non-carrier (wildtype) mice from the colony or to C57BL/6J inbred mice (Stock No. 000664).
Mating System	Noncarrier x Hemizygote         (Female x Male)   20-SEP-12
	Hemizygote x Noncarrier         (Female x Male)   20-SEP-12

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls

Control Information

	Control
	Noncarrier
	000664 C57BL/6J	(approximate)
Considerations for Choosing Controls
Control Pricing Information for Genetically Engineered Mutant Strains.

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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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