Strain Name:
B6;C3-Tg(Prnp-ITM2B/APP695*42)A12Emcg/Mmjax
Stock Number:
034842-JAX
Citation ID:
RRID:MMRRC_034842-JAX
Other Names:
This strain was formerly available as JaxMice stock number 7002. BRI-Abeta42A line 12e

Gene Information

Prnp
Name: prion protein
Synonyms: PrP, PrPC, Sinc, Prn-i, Prn-p, CD230, PrPSc
Type: Gene
Species: Mus musculus (mouse)
Chromosome: 2
NCBI: 19122
HGNC: HGNC:9449
Homologene: 7904
Tg(Prnp-ITM2B/APP695*42)A12Emcg
Name: transgene insertion A12, Eileen McGowan
Synonyms: RI-Abeta42A (12e), BRI-Abeta42A line 12e, BRI-Abeta42A, BRI-Abeta1-42
Type: Transgene
Species: Mus musculus (mouse)
Chromosome: unknown
Alteration at locus: Transgenic
Phenotype
Homozygous: Homozygote phenotype is expected to be similar to hemizygote phenotype.
Hemizygous: Mice hemizygous for this BRI-Abeta42 transgene are viable and fertile with a normal lifespan and no obvious behavioral abnormalities. Transgenic BRI-Abeta42 mRNA is expressed in a pattern characteristic of the mouse prion protein promoter; highest transgene expression levels are detected in the cerebellar granule cells and hippocampus, followed by the cortex, pons, thalamus, and midbrain. Wildtype BRI protein is cleaved by furin or a furin-like protease near the COOH-terminus which releases a soluble 23 amino acid peptide. In the transgenic fusion protein, Abeta1-42 is fused to the C terminus of the BRI protein at the furin-like cleavage site such that cleavage results in efficient Abeta1-42 secretion into the lumen or extracellular space. Therefore, these mice specifically express the Abeta1-42 isoform in the absence of human amyloid beta protein precursor (APP) overexpression. In contrast to BRI-Abeta40 transgenic mice, hemizygous BRI-Abeta42 mice accumulate detergent-insoluble amyloid-beta with age and develop cored plaques in the cerebellum at as early as three months of age. Development of forebrain pathology occurs later, and is more variable. Extracellular Abeta plaques are not present consistently in the hippocampus and entorhinal/piriform cortices until 12 months of age. BRI-Abeta42 mice also develop extensive congophillic amyloid angiopathy with age. These mutant mice may be useful in neurological studies of Alzheimer's disease, neurodegeneration, and amyloid plaque formation.
MeSH Terms
  • Age Factors
  • Amyloid beta-Peptides/genetics
  • Amyloid beta-Peptides/metabolism
  • Amyloid beta-Protein Precursor/genetics
  • Amyloid beta-Protein Precursor/metabolism
  • Animals
  • Blood Vessels/metabolism
  • Blood Vessels/pathology
  • Blood Vessels/ultrastructure
  • Blotting, Northern/methods
  • Blotting, Western/methods
  • Brain/metabolism
  • Brain/pathology
  • Brain/ultrastructure
  • Cerebral Amyloid Angiopathy/metabolism
  • Cerebral Amyloid Angiopathy/pathology
  • Enzyme-Linked Immunosorbent Assay/methods
  • Female
  • Glial Fibrillary Acidic Protein/metabolism
  • Humans
  • Immunohistochemistry/methods
  • Immunoprecipitation/methods
  • In Situ Hybridization/methods
  • Male
  • Mass Spectrometry/methods
  • Mice
  • Mice, Transgenic
  • Microscopy, Electron, Transmission/methods
  • Mutation
  • Peptide Fragments/analysis
  • Peptide Fragments/genetics
  • Peptide Fragments/metabolism
  • Pia Mater/metabolism
  • Pia Mater/pathology
  • Pia Mater/ultrastructure
  • Plaque, Amyloid/metabolism
  • Plaque, Amyloid/pathology
  • Thiazoles/metabolism
Strain Development
The BRI-Abeta42 transgene was generated with a mouse prion promoter upstream of a BRI-Abeta42 fusion construct. This fusion construct contained a cDNA sequence from human type 2 transmembrane protein (BRI or ITM2B) fused in-frame with a "wildtype APP695" cDNA sequence encoding amyloid-beta42 (Abeta42) at the furin-like cleavage site; the C-terminal 23 amino acid ABri peptide of BRI was replaced with the Abeta42 sequence. The transgene was injected into the pronuclei of single-cell embryos from C3B6F1 × B6 mice and then implanted into pseudopregnant females. Mice from the founder with the highest Abeta42 plasma levels, line BRI-Abeta42A (12e), were maintained on a mixed B6C3 background prior to arrival at The Repository.
Suggested Control Mice
Wild-ype littermates
  • Models for Human Disease
  • Neurobiology
Donor
Eileen McGowan, Ph.D., Mayo Clinic College of Medicine
Primary Reference
McGowan E, Pickford F, Kim J, Onstead L, Eriksen J, Yu C, Skipper L, Murphy MP, Beard J, Das P, Jansen K, Delucia M, Lin WL, Dolios G, Wang R, Eckman CB, Dickson DW, Hutton M, Hardy J, Golde T. Abeta42 is essential for parenchymal and vascular amyloid deposition in mice. Neuron. 2005 Jul 21;47(2):191-9 (Medline PMID: 16039562)

Colony and Husbandry Information

When maintaining a live colony, hemizygous mice can be bred with wildtype littermates or with B6C3F1/J.
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 csmmrrc@jax.org .

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 csmmrrc@jax.org 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.

The donor or their institution limits the distribution to non-profit institutions only.

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
034842-JAX-SPERM Cryo-preserved spermatozoa $437.00 / Non-Profit Aliquot Approximate quantity3
034842-JAX-RESUS Litter recovered from cryo-archive $2,022.00 / Non-Profit Litter Recovered litter4; additional fees for any special requests.
Cryopreserved material may be available upon request, please inquire to csmmrrc@jax.org 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.