Strain Name:

C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J

Stock Number:

016608

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TDP-43PrP transgenic mice express a full length wild type human TARDBP cDNA under control of a Prnp promoter. These transgenic mice may be useful in studying neuromuscular and neurodegenerative disorders such as ALS (Lou Gehrig's Disease).

Description

Strain Information

Former Names C57BL/6-Tg(Prnp-TARDBP)3cLtrc/J    (Changed: 02-FEB-12 )
C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J    (Changed: 01-JUN-11 )
Type Coisogenic; Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Mating SystemHemizygote x Hemizygote         (Female x Male)   17-OCT-11
Specieslaboratory mouse
GenerationF?+F3 (21-DEC-12)
Generation Definitions
 
Donating Investigator Leonard Petrucelli,   Mayo Clinic

Description
TDP-43PrP transgenic mice express a full length wild type human TAR DNA binding protein (TARDBP or TDP-43) cDNA under control of the prion protein (Prnp) promoter. Hemizygotes are viable, fertile, and normal in size. TDP-43 is a ubiquinated protein localized to the nucleus of cells. Accumulations of TDP-43 are involved in the development of Amyotrophic lateral sclerosis (ALS). These mice express wildtype human TDP-43 primarily in the nuclei of neurons throughout much of the gray matter of the spinal cord and brain, including the hippocampus, striatum, brainstem and cortex. Homozygotes and hemizygous express TDP-43 in brain at 2.5 and 1.9-fold endogenous TDP-43 levels, respectively. Endogenous TDP-43 is down-regulated in response to transgene overexpression. Beginning at 14 days of age, homozygous TDP-43PrP are smaller than hemizygous littermates. By 21 days of age homozygotes develop body tremors, have difficulty walking, and develop an aberrant "swimming" gait. Between 1 and 2 months of age they become unable to right themselves. These also mice exhibit reactive gliosis, and axonal and myelin degeneration. These mice exhibit truncated TDP-43 and ubiquitin aggregates containing phosphorylated TDP-43. Hemizygous mice are indistinguishable from wildtype littermates. These transgenic mice may be useful in studying neuromuscular and neurodegenerative disorders such as ALS (Lou Gehrig's Disease) and frontotemporal lobar degeneration with ubiquitin aggregates.

Development
The TDP-43PrP transgene was designed with the human TARDBP gene driven by a mouse prion protein (Prnp) promoter. The transgene was microinjected into fertilized C57BL/6 oocytes. Mice from the highest expressing founder line were bred to C57BL/6 mice to establish a colony. Upon arrival at The Jackson Laboratory, transgenic mice were bred to C57BL/6J mice (Stock No. 000664).

Control Information

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

Related Strains

View Amyotrophic Lateral Sclerosis (ALS)     (30 strains)

Strains carrying other alleles of Prnp
012938   129-Prnptm2Edin/J
016925   129;B6-Grin3b/Tmem259tm1Zhang Tg(Prnp-C19ORF6,-GFP)6Zhang/J
003960   129S6-Tg(Prnp-GFP/cre)1Blw/J
005866   B6.Cg-Tg(APP695)3Dbo Tg(PSEN1dE9)S9Dbo/Mmjax
006006   B6.Cg-Tg(Prnp-APP)A-2Dbo/J
008596   B6.Cg-Tg(Prnp-Abca1)EHol/J
006005   B6.Cg-Tg(Prnp-App/APPswe)E1-2Dbo/Mmjax
007180   B6.Cg-Tg(Prnp-ITM2B/APP695*40)1Emcg/J
007182   B6.Cg-Tg(Prnp-ITM2B/APP695*42)A12Emcg/J
006823   B6.Cg-Tg(Prnp-SNCA*A53T)23Mkle/J
010700   B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J
009337   B6.FVB-Tg(Prnp-RTN3)2Yanr/J
007002   B6;C3-Tg(Prnp-ITM2B/APP695*42)A12Emcg/Mmjax
008169   B6;C3-Tg(Prnp-MAPT*P301S)PS19Vle/J
004479   B6;C3-Tg(Prnp-SNCA*A53T)83Vle/J
018917   B6;SJL-Tg(Prnp-CCS)17Jlel/J
003378   B6C3-Tg(APP695)3Dbo Tg(PSEN1)5Dbo/J
008216   B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J
008075   B6CBA-Tg(Prnp-TBP*)105Xjl/J
008083   B6CBA-Tg(Prnp-TBP*)13Xjl/J
003741   B6D2-Tg(Prnp-MAPT)43Vle/J
024841   B6N.Cg-Tg(Prnp-MAPT*P301S)PS19Vle/J
017907   B6N.Cg-Tg(Prnp-TARDBP)96Dwc/J
017933   B6N.Cg-Tg(Prnp-TARDBP*Q331K)103Dwc/J
017930   B6N.Cg-Tg(Prnp-TARDBP*Q331K)109Dwc/J
016201   B6SJL-Tg(Prnp-TARDBP)4Jlel/J
016203   B6SJL-Tg(Prnp-TARDBP*A315T)23Jlel/J
017604   C57BL/6-Tg(Prnp-TARDBP*M337V)4Ptrc/J
019517   FVB-Tg(Prnp-HSPB1)1Kolb/J
019482   FVB-Tg(Prnp-HSPB1*R136W)1Kolb/J
018122   FVB.129S7(B6)-Prnptm1Cwe/J
017678   FVB;129-Pink1tm1Aub Tg(Prnp-SNCA*A53T)AAub/J
017744   FVB;129-Tg(Prnp-SNCA*A53T)AAub/J
017916   STOCK Tg(Prnp-FUS)WT3Cshw/J
016144   STOCK Tg(Prnp-TARDBP)4Jlel/J
016143   STOCK Tg(Prnp-TARDBP*A315T)23Jlel/J
008212   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(Prnp-SMN)92Ahmb/J
View Strains carrying other alleles of Prnp     (37 strains)

View Strains carrying other alleles of TARDBP     (13 strains)

Additional Web Information

Working with ALS Mice manual [.pdf]
This resource was prepared by scientists with Prize4Life and The Jackson Laboratory.

Visit the Amyotrophic Lateral Sclerosis (ALS) Mouse Model Resource site for helpful information on ALS Disease and research resources.

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.
Amyotrophic Lateral Sclerosis 10, with or without Frontotemporal Dementia;
Frontotemporal Lobar Degeneration with Tdp43 Inclusions, Grn-Related
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Tg(Prnp-TARDBP)3cPtrc/Tg(Prnp-TARDBP)3cPtrc

        C57BL/6-Tg(Prnp-TARDBP)3cPtrc
  • mortality/aging
  • premature death
    • mutants become moribound between 1 and 2 months of age   (MGI Ref ID J:163231)
  • growth/size/body phenotype
  • decreased body weight
    • body weight is reduced from 14 days of age onward   (MGI Ref ID J:163231)
  • behavior/neurological phenotype
  • abnormal gait
    • mutants develop an aberrant "swimming" gait   (MGI Ref ID J:163231)
    • by 21 days of age, mutants have difficulty walking faster than 10 cm/s and recruiting their hindlimbs   (MGI Ref ID J:163231)
  • impaired righting response
    • between 1 and 2 months of age, mutants are unable to right themselves   (MGI Ref ID J:163231)
  • limb grasping
    • mutants fail to show proper escape extension by splaying their hindlimbs upon elevation   (MGI Ref ID J:163231)
  • tremors
    • mutants exhibit body tremors by 21 days of age   (MGI Ref ID J:163231)
    • however, there is no evidence of muscle atrophy   (MGI Ref ID J:163231)
  • nervous system phenotype
  • abnormal brainstem morphology
    • in the brainstem, eosinphilic aggregates are found mostly within the pontine nuclei and midbrain tegmentum, including the red nucleus   (MGI Ref ID J:163231)
    • abnormal red nucleus morphology
      • eosinphilic aggregates   (MGI Ref ID J:163231)
  • abnormal motor neuron morphology
    • mutants exhibit neuronal cytoplasmic eosinophilic aggregates in spinal motor neurons   (MGI Ref ID J:163231)
    • about 10% of neurons in the anterior horn of the spinal cord had eosinophilic aggregates, with fewer in the posterior horn and brainstem   (MGI Ref ID J:163231)
    • motoneuron eosinophilic aggregates are composed of abnormal mitochondrial clusters; mitochondria have features suggestive of degeneration, with decreased cristae and vacuoles within the mitochondrial matrix   (MGI Ref ID J:163231)
  • abnormal spinal cord white matter morphology
    • degenerating mitochondria, and autophagic vacuoles, are seen within swollen axons in the spinal cord white matter   (MGI Ref ID J:163231)
  • axon degeneration
    • in the spinal cord   (MGI Ref ID J:163231)
  • decreased brain weight
    • brain weight is lower at 1 month of age   (MGI Ref ID J:163231)
  • demyelination
    • vacuolization of myelin and myelin ovoids indicate myelin degeneration in anterolateral funiculi of the spinal cord   (MGI Ref ID J:163231)
  • gliosis
    • reactive gliosis is seen in the anterior horn of the spinal cord   (MGI Ref ID J:163231)
    • astrocytosis
      • reactive astrocytosis is seen in the gray matter of the spinal cord   (MGI Ref ID J:163231)
  • neuron degeneration
    • degenerating neurites, axons and neurons are seen in the spinal cord   (MGI Ref ID J:163231)
View Research Applications

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

Neurobiology Research
Amyotrophic Lateral Sclerosis (ALS)
Ataxia (Movement) Defects
Myelination Defects
Neurodegeneration

Research Tools
Neurobiology Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(Prnp-TARDBP)3cPtrc
Allele Name transgene insertion 3c, Leonard Petrucelli
Allele Type Transgenic (random, expressed)
Common Name(s) TDP-43PrP; Tg(Prnp-TARDBP)3cLtrc;
Mutation Made By Leonard Petrucelli,   Mayo Clinic
Strain of OriginC57BL/6
Expressed Gene TARDBP, TAR DNA binding protein, human
Promoter Prnp, prion protein, mouse, laboratory
Molecular Note The TDP-43PrP transgene was designed with the human TAR DNA binding protein (TARDBP or TDP-43) gene driven by a mouse prion protein (Prnp) promoter. Line 3c was generated. [MGI Ref ID J:163231]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

Human TARDBP, QPCR
Tg(Prnp-TARDBP*A315T), Melt Curve Analysis
Tg(Prnp-TARDBP*A315T), Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Xu YF; Gendron TF; Zhang YJ; Lin WL; D'Alton S; Sheng H; Casey MC; Tong J; Knight J; Yu X; Rademakers R; Boylan K; Hutton M; McGowan E; Dickson DW; Lewis J; Petrucelli L. 2010. Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. J Neurosci 30(32):10851-9. [PubMed: 20702714]  [MGI Ref ID J:163231]

Additional References

Tg(Prnp-TARDBP)3cPtrc related

Xu YF; Prudencio M; Hubbard JM; Tong J; Whitelaw EC; Jansen-West K; Stetler C; Cao X; Song J; Zhang YJ. 2013. The pathological phenotypes of human TDP-43 transgenic mouse models are independent of downregulation of mouse Tdp-43. PLoS One 8(7):e69864. [PubMed: 23922830]  [MGI Ref ID J:203274]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           FGB27

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, these mice may be bred as hemizygotes.
Mating SystemHemizygote x Hemizygote         (Female x Male)   17-OCT-11
Diet Information LabDiet® 5K52/5K67

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 MaleHemizygous for Tg(Prnp-TARDBP)3cPtrc  
$232.00Female or MaleHomozygous for Tg(Prnp-TARDBP)3cPtrc  
Price per Pair (US dollars $)Pair Genotype
$464.00Hemizygous for Tg(Prnp-TARDBP)3cPtrc x Hemizygous for Tg(Prnp-TARDBP)3cPtrc  
$302.00Hemizygous for Tg(Prnp-TARDBP)3cPtrc x Noncarrier  
$302.00Noncarrier x Hemizygous for Tg(Prnp-TARDBP)3cPtrc  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

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 MaleHemizygous for Tg(Prnp-TARDBP)3cPtrc  
$301.60Female or MaleHomozygous for Tg(Prnp-TARDBP)3cPtrc  
Price per Pair (US dollars $)Pair Genotype
$603.20Hemizygous for Tg(Prnp-TARDBP)3cPtrc x Hemizygous for Tg(Prnp-TARDBP)3cPtrc  
$392.60Hemizygous for Tg(Prnp-TARDBP)3cPtrc x Noncarrier  
$392.60Noncarrier x Hemizygous for Tg(Prnp-TARDBP)3cPtrc  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

Control Information

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

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