Strain Name:
C3;B6-Tg(Prnp-ATN1)150Dbo/Mmmh
Stock Number:
000398-MU
Citation ID:
RRID:MMRRC_000398-MU

Strain Information

Prnp
Name: prion protein
Synonyms: PrP, PrPC, Prn-i, Sinc, Prn-p, CD230, PrPSc
Type: Gene
Species: Mouse
Chromosome: 2
NCBI: 19122
HGNC: HGNC:9449
Homologene: 7904
Atn1
Name: atrophin 1
Synonyms: atrophin-1, Atr1, Drpla
Type: Gene
Species: Mouse
Chromosome: 6
NCBI: 13498
HGNC: HGNC:3033
Homologene: 1461
Tg(Prnp-ATN1)150Dbo
Name: transgene insertion 150, David R Borchelt
Synonyms: AT-FL-65Q-150
Type: Transgene
Species: Mus musculus (mouse)
Chromosome: unknown
Alteration at locus: Transgenic
Genetic Alterations
Full-length atrophin-1 cDNAs (with no epitope tags) encoding 26 or 65 consecutive glutamines ([Q]n) were flanked with XhoI linkers and subcloned into the mouse prion protein promoter (Pro) vector (MoPrP.Xho). The noncoding portions of the second and third exons (E2 and E3) of the PrP gene were fused into one exon that contains the atrophin-1 cDNA insert (with its own start and stop codons).
Genotype Determination
Phenotype
Transgenic mice expressing full-length human atrophin-1 with 65 consecutive glutamines exhibit ataxia, retremors, abnormal movements, seizures, and premature death. These mice accumulate atrophin-1 immunoreactivity and inclusion bodies in the nuclei of multiple populations of neurons. Subcellular fractionation revealed 120 kDa nuclear fragments of mutant atrophin-1, whose abundance increased with age and phenotypic severity. Brains of DRPLA patients contained apparently identical 120 kDa nuclear fragments. By contrast, mice overexpressing atrophin-1 with 26 glutamines were phenotypically normal and did not accumulate the 120 kDa fragments.
MeSH Terms
  • Adolescent
  • Animals
  • Ataxia
  • Brain/pathology
  • Cell Nucleus/metabolism
  • Child
  • Chorea
  • Disease Models, Animal
  • Female
  • Humans
  • Male
  • Mice
  • Mice, Transgenic
  • Multiple System Atrophy/genetics
  • Multiple System Atrophy/metabolism
  • Multiple System Atrophy/pathology
  • Nerve Tissue Proteins/genetics
  • Nerve Tissue Proteins/metabolism
  • Neurodegenerative Diseases/genetics
  • Neurodegenerative Diseases/metabolism
  • Peptide Fragments/metabolism
  • Repetitive Sequences, Nucleic Acid
  • Tremor
Strain Development
DNA was injected into the male pronucleus of fertilized oocytes. Founders were analyzed for insertion of the transgene with a three-way PCR reaction with the following primers: PrP-sense (5'-CCTCTTTGTGAC TATGTGGACTGATGTCGG5–3') and PrP-antisense (5'-GTGGATAC CCCCTCCCCCAGCCTAGACC-3'), which together give a product of 700 bp, and At-3818–5' (5'-AGGTGGGGAGGTGGCGAGGAT-3'), which gives a transgene-specific product of 400 bp with PrP-antisense in transgenic animals. The copy number was established by probing of a Southern blot of genomic mouse tail DNA with random prime-labeled BamHI fragments of atrophin-1 (bp 2980–3348). Animals with more than ten copies were bred into stable transgenic lines. The lines were maintained on a hybrid C3H/B6 background
Suggested Control Mice
  • Wildtype littermates
MMRRC Genetic QC Summary
The MMRRC Centers have developed a genetic QC pipeline using MiniMUGA array genotyping to provide additional information on strain backgrounds for MMRRC congenic and inbred strains. For more information on when data may be available, or to request genotyping for a strain of interest, please contact mmrrc@missouri.edu. Older strains may not have this information.
  • Models for Human Disease
  • Neurobiology
  • Sensorineural
Donor
David R. Borchelt, Ph.D., Johns Hopkins University School of Medicine
Primary Reference
Schilling G, Wood JD, Duan K, Slunt HH, Gonzales V, Yamada M, Cooper JK, Margolis RL, Jenkins NA, Copeland NG, Takahashi H, Tsuji S, Price DL, Borchelt DR, Ross CA. Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA. Neuron. 1999 Sep;24(1):275-86. (Medline PMID: 10677044)

Colony and Husbandry Information

Colony Surveillance Program and Current Health Reports

Mice recovered from a cryo-archive will have health surveillance performed on recipient females. Health reports will be provided prior to shipment. If you require additional health status information, please email mmrrc@missouri.edu.

Order Request Information

Limited quantities of breeder mice (recovered litter) are available from a cryoarchive; recovered litter usually available to ship in 3 to 4 months.

Cryopreserved material may be available upon request, please inquire to mmrrc@missouri.edu for more information.

Distribution of this strain requires submission of the MMRRC Conditions of Use (COU). A link to the COU web form will be provided via email after an order has been placed; the form should be completed then or the email forwarded to your institutional official for completion.

Additional charges may apply for any special requests. Shipping costs are in addition to the basic distribution/resuscitation fees. Information on shipping costs and any additional charges will be provided by the supplying MMRRC facility.

Click button to Request this one strain. (Use the MMRRC Catalog Search to request more than one strain.)
MMRRC Item # Description Distribution Fee / Unit (US $)
*Shipping & Handling not included*
Units Notes
000398-MU-SPERM Cryo-preserved spermatozoa $437.00 / $817.00
Non-Profit / For-Profit
Aliquot Approximate quantity3
000398-MU-RESUS Litter recovered from cryo-archive $2,016.00 / $4,103.00
Non-Profit / For-Profit
Litter Recovered litter4; additional fees for any special requests.
Cryopreserved material may be available upon request, please inquire to mmrrc@missouri.edu for more information.

1 The distribution fee covers the expense of rederiving mice from a live mouse; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

2 An aliquot contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

3 An aliquot is one straw or vial with sufficient sperm to recover at least one litter of mice, as per provided protocols, when performed at the MMRRC facility. The MMRRC makes no guarantee concerning the success of these procedures when performed outside the MMRRC facilities.

4 The distribution fee covers the expense of resuscitating mice from the cryo-archive; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.