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Availability & Fees Order this Strain
A targeting vector was designed to insert the R236H mutation in exon 4 (corresponding to the R239H mutation in humans), which is found in Alexander Disease patients. Cre-mediated recombination with a nestin-cre removed the floxed neo cassette.
Homozygous: Not characterized
Heterozygous: The mice form protein aggregates known as Rosenthal fibers in astrocytes throughout the central nervous system. The mice display abnormal response to seizure inducing agent. The mice display deficits in learning and memory.
Correctly targeted AB-1 ES cells derived from 129S7/SvEvBrd were injected into C57BL/6J blastocysts. Chimeric founders were bred to Rosa26-cre mice (likely carrying Tg(Gt(ROSA)26Sor-cre)15Eps - MGI:3715365) in unconfirmed FVB background to delete the floxed neo-cassette. Offspring were subsequently backcrossed to C57BL/6J for over 20 generations and maintained as heterozygotes. This mutation is also available on a FVB/NTac background, MMRRC:037369, FVB.129-Gfaptm2Mes/Mmnc.
Hagemann TL, Connor JX, Messing A. Alexander disease-associated GFAP mutations in mice induce Rosenthal fiber formation and a white matter stress response. J Neurosci. 2006 Oct 25;26(43):11162-73. (Medline PMID: 17065456)
Colony Surveillance Program and Current Health Reports
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.
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.
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