Strain Name:

B6SJL-Tg(Prnp-TARDBP)4Jlel/J

Stock Number:

016201

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TDP43 transgenic mice express a full-length human TAR DNA binding protein (TARDBP) cDNA under the control of mouse prion protien promoter. 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.

Description

Strain Information

Former Names B6SJL.Cg-Tg(Prnp-TARDBP)4Jlel/J    (Changed: 28-MAY-12 )
Type Congenic; Mutant Stock; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Mating SystemHemizygote x F1         (Female x Male)   01-MAR-12
Mating SystemF1 x Hemizygote         (Female x Male)   01-MAR-12
Specieslaboratory mouse
GenerationF?+N6 (11-DEC-13)
Generation Definitions
 
Donating Investigator Jeffrey L Elliott,   The University of Texas Southwestern Medical Center

Description
TDP43 transgenic mice express a full length human TAR DNA binding protein (TARDBP or TDP-43) cDNA inserted between exon 2 and exon 3 of the mouse prion protein (Prnp) gene. The transgene in founder line 4 was later found to have integrated on the X chromosome. Hemizygotes are viable and fertile, with mean survival of 109 days. TDP-43 is a ubiquinated protein localized to the nucleus of nerve cells. Accumulations of mutated TDP-43 are involved in the development of Amyotrophic lateral sclerosis (ALS). These mice express human TDP-43 in the spinal cord and skeletal muscle. They develop a late onset progressive motor phenotype, characterized by bilateral proximal weakness, spasticity, reduced spontaneous movements and progressive weight loss. These mice contain a nuclear localized, less truncated form of TDP-43 than that observed in TDP-43*A315T mice (Stock No. 016203). Disease progression is later than that observd in TDP-43*A315T mice. 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.

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.

Development
A full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence was inserted between exon 2 and exon 3 of mouse prion protein (PrP or Prnp) gene at two unique XhoI sites in the Mo-Prp.Xho plasmid vector (ATCC#JHU-2). The resulting transgene was microinjected into B6SJLF1 oocytes and mice from founder line 4, in which the transgene integrated on the X chromosome, were bred with CD1 mice to establish a colony. The donating investigator maintained these mice on a mixed background. Upon arrival at The Jackson Laboratory, transgenic mice were bred with B6SJLF1/J mice (Stock No. 100012) for at least 5 generations to establish the colony.

Control Information

  Control
   Noncarrier
   100012 B6SJLF1/J (approximate)
 
  Considerations for Choosing Controls

Related Strains

View Amyotrophic Lateral Sclerosis (ALS)     (30 strains)

Strains carrying   Tg(Prnp-TARDBP)4Jlel allele
016144   STOCK Tg(Prnp-TARDBP)4Jlel/J
View Strains carrying   Tg(Prnp-TARDBP)4Jlel     (1 strain)

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
016203   B6SJL-Tg(Prnp-TARDBP*A315T)23Jlel/J
016608   C57BL/6-Tg(Prnp-TARDBP)3cPtrc/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
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     (36 strains)

View Strains carrying other alleles of TARDBP     (12 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;
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Tg(Prnp-TARDBP)4Jlel/0

        involves: C57BL/6 * CD-1 * SJL
  • mortality/aging
  • premature death
    • mean survival is 109 days   (MGI Ref ID J:165985)
  • muscle phenotype
  • hypotonia   (MGI Ref ID J:165985)
  • increased variability of skeletal muscle fiber size   (MGI Ref ID J:165985)
  • muscle weakness
    • mice exhibit weakness that manifests as external rotation in one hindlimb followed by development of severe bilateral proximal weakness and hypotonia unlike wild-type mice   (MGI Ref ID J:165985)
  • myopathy
    • muscles exhibit variation in muscle fiber size, prominent myopathic grouping, and disorganization of the myofiber internal architecture unlike wild-type muscles   (MGI Ref ID J:165985)
  • behavior/neurological phenotype
  • abnormal motor coordination/ balance
    • mice exhibit late onset, progressive motor defects compared with wild-type mice   (MGI Ref ID J:165985)
  • decreased grip strength   (MGI Ref ID J:165985)
  • short stride length   (MGI Ref ID J:165985)
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
Neurodegeneration

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(Prnp-TARDBP)4Jlel
Allele Name transgene insertion 4, Jeffrey L Elliott
Allele Type Transgenic (random, expressed)
Common Name(s) WT TDP-43 line 4;
Mutation Made By Jeffrey Elliott,   The University of Texas Southwestern Medical Center
Strain of Origin(C57BL/6 x SJL)F1
Expressed Gene TARDBP, TAR DNA binding protein, human
Promoter Prnp, prion protein, mouse, laboratory
Molecular Note The mouse promoter drives neuronal expression of the wild-type human cDNA. Several lines were established (including 4, 13, and 21). [MGI Ref ID J:165985]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

** human TARDBP*A315T, Pyrosequencing
Tg(Prnp-TARDBP), High Resolution Melting
Tg(Prnp-TARDBP),

Separated MCA


Tg(Prnp-TARDBP), Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Stallings NR; Puttaparthi K; Luther CM; Burns DK; Elliott JL. 2010. Progressive motor weakness in transgenic mice expressing human TDP-43. Neurobiol Dis 40(2):404-14. [PubMed: 20621187]  [MGI Ref ID J:165985]

Additional References

Tg(Prnp-TARDBP)4Jlel related

Stallings NR; Puttaparthi K; Dowling KJ; Luther CM; Burns DK; Davis K; Elliott JL. 2013. TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis. PLoS One 8(8):e71793. [PubMed: 23967244]  [MGI Ref ID J:206353]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           MGL375

Colony Maintenance

Breeding & HusbandryMutant mice were bred to B6SJLF1/J hybrid mice for many generations to establish this strain. When maintaining the live colony, hemizygous mice are bred with B6SJLF1/J hybrid mice (Stock No. 100012). The transgene in this founder line was found to have integrated on the X chromosome. On a mixed genetic background (CD1;C57BL/6;SJL),transgenic mice only survive up to 109 days with decreased motor function and progressive neurodegeneration.
Mating SystemHemizygote x F1         (Female x Male)   01-MAR-12
F1 x Hemizygote         (Female x Male)   01-MAR-12

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)4Jlel  
Price per Pair (US dollars $)Pair Genotype
$254.95B6SJLF1/J (100012) x Hemizygous for Tg(Prnp-TARDBP)4Jlel  
$253.45Hemizygous for Tg(Prnp-TARDBP)4Jlel x B6SJLF1/J (100012)  

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)4Jlel  
Price per Pair (US dollars $)Pair Genotype
$331.50B6SJLF1/J (100012) x Hemizygous for Tg(Prnp-TARDBP)4Jlel  
$329.50Hemizygous for Tg(Prnp-TARDBP)4Jlel x B6SJLF1/J (100012)  

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

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