Strain Detail Sheet

Strain Name    :

B6.129S1-Uchl3tm1Tilg/Mmmh

Stock Number :

010587-MU

Gene Information

Gene Details [Including genotyping protocols]

(provided by MGI)
Allele Symbol: Uchl3tm1Tilg
Name: targeted mutation 1, Shirley M Tilghman
Alteration at locus: Knockout
Gene Symbol: Uchl3
Name: ubiquitin carboxyl-terminal esterase L3 (ubiquitin thiolesterase)
Chromosome: 14
Alteration at locus: Knockout

Genetic Alterations:
Exons 3 through 7 were deleted using a targeting deletion construct containing the neomycin resistance marker.

Genotype Determination:

ES Cell Line: CJ7 derived from 129S1/Sv-Oca2+ Tyr+ Kitl+

Strain Description [Including phenotype, strain background, strain development and suggested control mice]

Phenotype

Homozygous phenotype: Homozygous mice breed well. They have no overt developmental abnormalities (Kurihara et al., 2000). However, donor submitted a manuscript that has been accepted for publication in "Hippocampus" demonstrating that UchL3 knockout mice exhibit working memory impairments.

Heterozygous phenotype: Heterozygous mice have no overt phenotype.


Mammalian Phenotype Terms:(provided by MGI)      Extend all MPTs
      assigned by genotype

The following phenotype information may relate to one or more alleles on a genetic background differing from this MMRRC strain.
Uchl3tm1Tilg/Uchl3tm1Tilg
        involves: 129S1/Sv * C57BL/6
  • vision/eye phenotype
    • abnormal retinal layer morphology (MGI Ref ID J:83810)
      • the distal photoreceptor region is dramatically shortened
      • abnormal retinal outer nuclear layer morphology (MGI Ref ID J:83810)
        • mice show an indistinguishable retinal phenotype to Lmo7tm1Tilg with dramatically reduced cell numbers in this layer
  • muscle phenotype
    • abnormal skeletal muscle fiber morphology (MGI Ref ID J:83810)
      • mice show similar defects to Lmo7tm1Tilg mice with increased nuclei number and inclusions, but with a lower penetrance and later onset; nuclear defects are observed in 5/9 homozygotes at 170 days after birth compared to 6/6 Lmo7-null mice at 80 days of age
  • growth/size phenotype
    • decreased body weight (MGI Ref ID J:71819)
      • weight is 20% less than wild-type by 80 days of age
  • nervous system phenotype
    • abnormal dorsal root ganglion morphology (MGI Ref ID J:71819)
      • 3.6-fold increase in small basophilic DRG cell bodies
    • axonal dystrophy (MGI Ref ID J:71819)
  • immune system phenotype
    • immune system phenotype (MGI Ref ID J:61093)
      • initial investigation of thymi show no significant differences from wild-type
  • reproductive system phenotype
    • reproductive system phenotype (MGI Ref ID J:61093)
      • initial investigation of testes show no significant differences from wild-type
Uchl3tm1Tilg/Uchl3tm1Tilg
        involves: 129S1/Sv * C57BL/6J
  • vision/eye phenotype
    • abnormal photoreceptor inner segment morphology (MGI Ref ID J:110169)
      • mitochondria at the inner segment are swollen; percentage of cristae to total area of mitochondrion in the inner segment is lower than in wild-type mice
    • abnormal photoreceptor outer segment morphology (MGI Ref ID J:110169)
      • a significant decrease in thickness by 6 weeks of age is observed
    • abnormal retinal apoptosis (MGI Ref ID J:110169)
      • at 3, 6, and 8 weeks of age, numbers of apoptotic cells are increased at the outer layer in the retina compared to wild-type
    • abnormal retinal outer nuclear layer morphology (MGI Ref ID J:110169)
      • chromatin condensation in photoreceptor nuclei in sometimes observed in the outer nuclear layer at 3 weeks of age
      • thin retinal outer nuclear layer (MGI Ref ID J:110169)
        • layer is significantly decreased in thickness by 6 weeks of age
    • abnormal retinal vasculature morphology (MGI Ref ID J:110169)
    • retinal degeneration (MGI Ref ID J:110169)
      • the retinal begins to degenerate at 3 weeks of age in the inner segment and ultimately disappears by 12 weeks
    • thin retinal outer plexiform layer (MGI Ref ID J:110169)
      • layer is significantly decreased in thickness by 6 weeks of age
  • cardiovascular system phenotype
    • abnormal retinal vasculature morphology (MGI Ref ID J:110169)
  • nervous system phenotype
    • abnormal photoreceptor inner segment morphology (MGI Ref ID J:110169)
      • mitochondria at the inner segment are swollen; percentage of cristae to total area of mitochondrion in the inner segment is lower than in wild-type mice
    • abnormal photoreceptor outer segment morphology (MGI Ref ID J:110169)
      • a significant decrease in thickness by 6 weeks of age is observed

Strain of Origin: C57BL/6

Strain genetic background: C57BL/6

Strain Development: The desicription of this strain's development is described in: Kurihara LJ, Semenova E, Levorse JM, Tilghman SM. Expression and functional analysis of Uch-L3 during mouse development. Mol Cell Biol. 2000 Apr;20(7):2498-504. (Medline PMID: 10713173)
Dr. Abel's laboratory maintained these mice on C57BL/6 for at least ten generations.

Suggested Control Mice:

  • Wildtype littermates

Research Applications

  • Models for Human Disease
  • Neurobiology
  • Learning and Memory

Strain Origin

Donor:

Primary Reference:

  • Kurihara LJ, Semenova E, Levorse JM, Tilghman SM. Expression and functional analysis of Uch-L3 during mouse development. Mol Cell Biol. 2000 Apr;20(7):2498-504. (Medline PMID: 10713173)
  • Kurihara LJ, Kikuchi T, Wada K, Tilghman SM. Loss of Uch-L1 and Uch-L3 leads to neurodegeneration, posterior paralysis and dysphagia. Hum Mol Genet. 2001 Sep 1;10(18):1963-70. (Medline PMID: 11555633 )
  • MA Wood, MP Kaplan, CM Brensinger, W Guo, and T Abel. Ubiquitin C-terminal hydrolase L3 (UchL3) is involved in working memory. Hippocampus. IN PRESS.

Colony and Husbandry Information

Special Considerations

None

Health Status Report

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

Availability Level

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

Conditions of Distribution [Including applicable technology transfer agreements]

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.

Fees

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 $)
Units Notes
010587-MU-RESUSLitter recovered from cryo-archive
$2,022.00
Non-Profit
Litter Recovered litter1; additional fees for any special requests.

1 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.

2 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.

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

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 above). 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.



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.



The MMRRC is a collaborative effort, funded by grants from DPCPSI of the NIH.

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