Strain Name:

B6(SJL)-Tardbptm1.1Pcw/J

Stock Number:

017591

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Availability:

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These floxed mutant mice possess loxP sites flanking exon 3 of the Tardbp gene. This strain may be useful for generating conditional mutations in applications related to studies of amyotrophic lateral sclerosis (ALS).

Description

Strain Information

Type Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Mating SystemHomozygote x Homozygote         (Female x Male)   11-FEB-14
Specieslaboratory mouse
GenerationpN1F3 (20-AUG-14)
Generation Definitions
 
Donating Investigator Philip C. Wong,   Johns Hopkins University

Description
These TardbpF mutant mice possess loxP sites flanking exon 3 of the TAR DNA binding protein gene (Tardbp; TDP-43). The Tardbp gene product, TDP-43, is a protein localized to the nucleus of nerve cells. Ubiquinated accumulations of mutant TDP-43 have been linked to the development of Lou Gehrig's disease (Amyotrophic lateral sclerosis; ALS) and frontotemporal lobar degeneration. Mice that are homozygous for this allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. When bred to mice that express Cre recombinase, resulting offspring will have exon 3 deleted in the cre-expressing tissues.

For example, when bred to B6;129-Gt(ROSA)26Sortm1(cre/ERT)Nat/J (Stock No. 004847), tamoxifen-induced Cre-mediated recombination results in death 9 days after induction in the offspring. These mice exhibit dramatic loss of white and brown fat due to decrease in food consumption and increased fat metabolism. These mice also display decrease in the expression of TBC1 domain family, member 1 (Tbc1d1) gene, which has been linked to leanness, human obesity, and glucose transport in skeletal muscle.

When bred to mice carrying Tg(CAG-(cre/Esr1*)5Amc (Stock No. 004682), tamoxifen-induced Cre-mediated recombination results in loss of body fat and altered fat cell morphology.

Development
A targeting vector was designed to insert a loxP site upstream of exon 3 followed by a frt-flanked neomycin resistance (neo) cassette, and a second loxP site downstream of exon 3 of the TAR DNA binding protein (Tardbp) gene. The construct was electroporated into C57BL/6-derived V26.2 embryonic stem (ES) cells, and correctly targeted ES cells were injected into B6(Cg)-Tyrc-2J/J blastocysts. Resulting chimeric mice were bred with Bmpr1bTg(ACTB-FLP)4917Dym transgenic mice (on a B6SJL background) to delete the neo cassette, and progeny were crossed to remove the Flp-expressing transgene. Upon arrival, mice were bred to C57BL/6J inbred mice (Stock No. 000664) for at least one generation to establish the colony.

Control Information

  Control
   100012 B6SJLF1/J (approximate)
   000664 C57BL/6J (approximate)
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Tardbp
019899   B6(C3H)-Tardbpm1H/J
012850   B6;129P2-TardbpGt(RRB030)Byg/J
View Strains carrying other alleles of Tardbp     (2 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Amyotrophic Lateral Sclerosis 10, with or without Frontotemporal Dementia;   (TARDBP)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype relates to a compound genotype created using this strain.
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Gt(ROSA)26Sortm1(cre/ERT)Nat/Gt(ROSA)26Sor+ Tardbptm1.1Pcw/Tardbp+

        involves: 129 * C57BL/6 * SJL   (conditional)
  • growth/size/body phenotype
  • decreased body weight
    • during the initial 3 days after cre induction by tamoxifen, all mice show decrease in body weight due to decreased food intake, but whereas controls regain recover some weight, experimental mice do not   (MGI Ref ID J:164406)
  • decreased total body fat amount
    • inspection of animals dying after cre induction by tamoxifen shows loss of body fat in these mice   (MGI Ref ID J:164406)
    • analyses of carcasses show significant decreases in whole body fat mass, but not lean mass   (MGI Ref ID J:164406)
  • homeostasis/metabolism phenotype
  • abnormal respiratory quotient
    • following cre induction, animals exhibit a decreased respiratory exchange ratio (RER) indicative of pure fat oxidation compared to controls by day 6   (MGI Ref ID J:164406)
  • adipose tissue phenotype
  • decreased total body fat amount
    • inspection of animals dying after cre induction by tamoxifen shows loss of body fat in these mice   (MGI Ref ID J:164406)
    • analyses of carcasses show significant decreases in whole body fat mass, but not lean mass   (MGI Ref ID J:164406)

Gt(ROSA)26Sortm1(cre/ERT)Nat/Gt(ROSA)26Sor+ Tardbptm1.1Pcw/Tardbptm1.1Pcw

        involves: 129 * C57BL/6 * SJL   (conditional)
  • mortality/aging
  • abnormal survival
    • when cre expression is induced with a tamoxifen-containing diet (400 mg/kg of chow), mice die by day 9 post-introduction of tamoxifen   (MGI Ref ID J:164406)
  • growth/size/body phenotype
  • decreased body weight
    • during the initial 3 days after cre induction by tamoxifen, all mice show decrease in body weight due to decreased food intake, but whereas controls regain recover some weight, experimental mice do not   (MGI Ref ID J:164406)
    • weight loss
      • mice show a cumulative loss of body weight compared to controls following cre induction, but energy intake (cumulative food intake) is similar to controls   (MGI Ref ID J:164406)
  • decreased total body fat amount
    • inspection of animals dying after cre induction by tamoxifen shows loss of body fat in these mice   (MGI Ref ID J:164406)
    • analyses of carcasses show significant decreases in whole body fat mass, but not lean mass   (MGI Ref ID J:164406)
  • homeostasis/metabolism phenotype
  • *normal* homeostasis/metabolism phenotype
    • without induction of cre by tamoxifen treatment, animals are indistinguishable from controls   (MGI Ref ID J:164406)
    • abnormal lipid oxidation
      • increased fat oxidation after induction is basis for increased weight loss   (MGI Ref ID J:164406)
    • abnormal respiratory quotient
      • following cre induction, animals exhibit a decreased respiratory exchange ratio (RER) indicative of pure fat oxidation compared to controls by day 6   (MGI Ref ID J:164406)
  • adipose tissue phenotype
  • decreased total body fat amount
    • inspection of animals dying after cre induction by tamoxifen shows loss of body fat in these mice   (MGI Ref ID J:164406)
    • analyses of carcasses show significant decreases in whole body fat mass, but not lean mass   (MGI Ref ID J:164406)
  • cellular phenotype
  • abnormal lipid oxidation
    • increased fat oxidation after induction is basis for increased weight loss   (MGI Ref ID J:164406)
  • behavior/neurological phenotype
  • abnormal food intake
    • energy intake on day 7 is significantly higher than on day 1 after cre induction   (MGI Ref ID J:164406)

Tardbptm1.1Pcw/Tardbp+ Tg(CAG-cre/Esr1*)5Amc/0

        involves: 129 * C57BL/6 * CBA * SJL   (conditional)
  • growth/size/body phenotype
  • decreased body weight
    • after cre induction by tamoxifen, mice show decrease in body weight relative to controls   (MGI Ref ID J:164406)
  • adipose tissue phenotype
  • abnormal brown fat cell morphology
    • absence of fatty acid vacuoles in adipocytes in interscapular brown fat is detected   (MGI Ref ID J:164406)
  • abnormal white fat cell morphology
    • reduction of fatty acid vacuoles in adipocytes in subcutaneous fat is detected   (MGI Ref ID J:164406)

Tardbptm1.1Pcw/Tardbptm1.1Pcw Tg(CAG-cre/Esr1*)5Amc/0

        involves: 129 * C57BL/6 * CBA * SJL   (conditional)
  • growth/size/body phenotype
  • decreased body weight
    • after cre induction by tamoxifen, mice show decrease in body relative to controls   (MGI Ref ID J:164406)
  • decreased total body fat amount
    • inspection of animals dying after cre induction by tamoxifen shows loss of body fat in these mice   (MGI Ref ID J:164406)
    • analyses of carcasses show significant decreases in whole body fat mass, but not lean mass   (MGI Ref ID J:164406)
  • cellular phenotype
  • abnormal lipid oxidation
    • increased fat oxidation after loss of Tardbp results in fat loss in adipocytes   (MGI Ref ID J:164406)
  • adipose tissue phenotype
  • abnormal brown fat cell morphology
    • absence of fatty acid vacuoles in adipocytes in interscapular brown fat is detected   (MGI Ref ID J:164406)
    • adipocytes are present in brown fat, but lack of stored fat within adipocytes results in loss of fat content in animals after induction by tamoxifen   (MGI Ref ID J:164406)
  • abnormal white adipose tissue morphology   (MGI Ref ID J:164406)
    • abnormal white fat cell morphology
      • reduction of fatty acid vacuoles in adipocytes in subcutaneous fat is detected   (MGI Ref ID J:164406)
      • adipocytes are present in white fat, but lack of stored fat within adipocytes results in loss of fat content in animals after induction by tamoxifen   (MGI Ref ID J:164406)
  • decreased mesenteric fat pad weight
    • dramatic fat loss is observed   (MGI Ref ID J:164406)
  • decreased total body fat amount
    • inspection of animals dying after cre induction by tamoxifen shows loss of body fat in these mice   (MGI Ref ID J:164406)
    • analyses of carcasses show significant decreases in whole body fat mass, but not lean mass   (MGI Ref ID J:164406)
  • homeostasis/metabolism phenotype
  • abnormal lipid oxidation
    • increased fat oxidation after loss of Tardbp results in fat loss in adipocytes   (MGI Ref ID J:164406)
View Research Applications

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

Cardiovascular Research
Other
      altered fat metabolism

Developmental Biology Research
Postnatal Lethality
      Homozygous

Endocrine Deficiency Research
Adipose Defects

Internal/Organ Research
Adipose Defects

Neurobiology Research
Amyotrophic Lateral Sclerosis (ALS)
Cre-lox System
      loxP-flanked Sequences
Metabolic Defects

Research Tools
Cre-lox System
      loxP-flanked Sequences
Metabolism Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tardbptm1.1Pcw
Allele Name targeted mutation 1.1, Philip C Wong
Allele Type Targeted (Conditional ready (e.g. floxed), No functional change)
Common Name(s) TardbpF;
Mutation Made By Philip Wong,   Johns Hopkins University
Strain of OriginC57BL/6
Gene Symbol and Name Tardbp, TAR DNA binding protein
Chromosome 4
Gene Common Name(s) 1190002A23Rik; ALS10; C85084; RIKEN cDNA 1190002A23 gene; TDP-43; Tdp43; expressed sequence C85084;
Molecular Note Exon 3 of the Tardbp gene was flanked by loxP sites. A neo cassette was inserted into exon 2. Mice carrying the mutant allele were crossed to Flp transgenic mice to delete the neo cassette. [MGI Ref ID J:164406]

Genotyping

Genotyping Information

Genotyping Protocols

Tardbptm1.1Pcw, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Chiang PM; Ling J; Jeong YH; Price DL; Aja SM; Wong PC. 2010. Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism. Proc Natl Acad Sci U S A 107(37):16320-4. [PubMed: 20660762]  [MGI Ref ID J:164406]

Additional References

Tardbptm1.1Pcw related

Haidet-Phillips AM; Gross SK; Williams T; Tuteja A; Sherman A; Ko M; Jeong YH; Wong PC; Maragakis NJ. 2013. Altered astrocytic expression of TDP-43 does not influence motor neuron survival. Exp Neurol 250:250-9. [PubMed: 24120466]  [MGI Ref ID J:206607]

Shan X; Chiang PM; Price DL; Wong PC. 2010. Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice. Proc Natl Acad Sci U S A 107(37):16325-30. [PubMed: 20736350]  [MGI Ref ID J:164382]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX12

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, homozygous mice may be bred together.
Mating SystemHomozygote x Homozygote         (Female x Male)   11-FEB-14
Diet Information LabDiet® 5K20

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $232.00Female or MaleHomozygous for Tardbptm1.1Pcw  
Price per Pair (US dollars $)Pair Genotype
$464.00Homozygous for Tardbptm1.1Pcw x Homozygous for Tardbptm1.1Pcw  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $301.60Female or MaleHomozygous for Tardbptm1.1Pcw  
Price per Pair (US dollars $)Pair Genotype
$603.20Homozygous for Tardbptm1.1Pcw x Homozygous for Tardbptm1.1Pcw  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Control Information

  Control
   100012 B6SJLF1/J (approximate)
   000664 C57BL/6J (approximate)
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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