Strain Name:

STOCK Tg(Prnp-TARDBP*A315T)23Jlel/J

Stock Number:

016143

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TDP43A315T transgenic mice express a full length human TAR DNA binding protein cDNA sequence bearing an A315T amino acid substitution, directed by a mouse prion protein promoter. These transgenic mice may be useful in applications related to the study of neuromuscular and neurodegenerative disorders such as ALS (Amyotrophic lateral sclerosis, Lou Gehrig's Disease) and frontotemporal lobar degeneration with ubiquitin aggregates.

Description

Strain Information

Type Mutant Stock; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Mating SystemNoncarrier x Hemizygote         (Female x Male)   03-FEB-12
Mating SystemHemizygote x Noncarrier         (Female x Male)   03-FEB-12
Specieslaboratory mouse
Generation?+N1F1 (21-AUG-14)
Generation Definitions
 
Donating Investigator Jeffrey L Elliott,   The University of Texas Southwestern Medical Center

Description
TDP43A315T transgenic mice express a full-length human TAR DNA binding protein (TARDBP) cDNA containing an A315T amino acid substitution. Expression is directed by the mouse prion protein (Prnp) promoter. The donating investigator stated that hemizygotes from founder line 23 are fertile, with a mean survival of 75 days (see survival note below). In neurons of patients with Amyotrophic lateral sclerosis (ALS), ubiquitin and TDP43 positive inclusions are observed. These mice express high levels of A315T TDP-43 in spinal cord, brainstem and brain. Specifically, levels in spinal cord are four-fold that of levels seen in non-transgenic mice. They develop a late onset progressive motor phenotype, characterized by hind limb weakness, progressive decline in grip strength, and reduction in stride length. A shift to cytosolic localization of A315T TDP-43 as well as lower molecular weight form of TDP-43 leads to earlier disease progression in these mice than in WT TDP-43 mice (Stock No. 016144). These transgenic mice may be useful in studying neuromuscular and neurodegenerative disorders such as ALS (Amyotrophic lateral sclerosis, Lou Gehrig's Disease) and frontotemporal lobar degeneration with ubiquitin aggregates.

An increase in mean survival was seen in a colony of TDP43A315T transgenic mice housed at The Jackson Laboratory. The data shows that hemizygous males have median survival of 109 days, while hemizygous females have median survival of 164 days.

Development
A full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence was modified to harbor the A315T amino acid substitution associated with familial ALS. This TDP43A315T 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 Prp-TDP43A315T transgene was microinjected into B6SJLF1 oocytes and mice from founder line 23 were bred with CD1 mice to establish a TDP43A315T colony. These mice were maintained on a mixed background. Upon arrival at The Jackson Laboratory, transgenic mice were bred with non-transgenic littermates to establish the colony.

Control Information

  Control
   Noncarrier
 
  Considerations for Choosing Controls

Related Strains

View Amyotrophic Lateral Sclerosis (ALS)     (36 strains)

Strains carrying   Tg(Prnp-TARDBP*A315T)23Jlel allele
016203   B6SJL-Tg(Prnp-TARDBP*A315T)23Jlel/J
View Strains carrying   Tg(Prnp-TARDBP*A315T)23Jlel     (1 strain)

Strains carrying other alleles of Prnp
012938   129-Prnptm2Edin/J
016925   129;B6-Del(10Grin3b-Tmem259)1Zang 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
025402   B6SJL-Tg(Prnp-Immt/SOD1)1Gmnf/J
025403   B6SJL-Tg(Prnp-Immt/SOD1*G93A)7Gmnf/J
016201   B6SJL-Tg(Prnp-TARDBP)4Jlel/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
016144   STOCK Tg(Prnp-TARDBP)4Jlel/J
008212   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(Prnp-SMN)92Ahmb/J
View Strains carrying other alleles of Prnp     (38 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*A315T)23Jlel/0

        involves: C57BL/6 * CD-1 * SJL
  • mortality/aging
  • premature death
    • mean survival is 75 days   (MGI Ref ID J:165985)
  • nervous system phenotype
  • abnormal brainstem morphology
    • brainstem neurons exhibit ubiquitin inclusions and astrogliosis unlike in wild-type mice   (MGI Ref ID J:165985)
  • abnormal spinal cord ventral horn morphology
    • mice exhibit marked astrogliosis compared with wild-type mice   (MGI Ref ID J:165985)
    • ventral horn neurons exhibit prominent ubiquitin positive cytoplasmic inclusions unlike in wild-type mice   (MGI Ref ID J:165985)
    • however, no pale amorphous cyto-architectural abnormalities observed in early onset severe founders that fail to exhibit germline transmission   (MGI Ref ID J:165985)
  • astrocytosis
    • in the ventral horn and brainstem neurons   (MGI Ref ID J:165985)
  • neuronal intranuclear inclusions
    • rare with TARDBP co-localization   (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
  • short stride length
  • muscle phenotype
  • abnormal quadriceps morphology
    • quadriceps muscle exhibits atrophy and angular fibers consistent with denervation changes compared with wild-type mice   (MGI Ref ID J:165985)
  • muscle weakness
    • in the hindlimb   (MGI Ref ID J:165985)
  • skeletal muscle atrophy
    • in the quadriceps muscle   (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*A315T)23Jlel
Allele Name transgene insertion 23, Jeffrey L Elliott
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) A315T TDP-43 line 23;
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 human cDNA with point mutations that result in the amino acid substitution of threonine for alanine at position 315 (A315T). This mutation was identified in human patients with amyotrophic lateral sclerosis with TARDBP proteinopathies. Four lines were established (23, 27, 35, and 61). [MGI Ref ID J:165985]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

** human TARDBP*A315T, Pyrosequencing
Tg(Prnp-TARDBP), High Resolution Melting
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*A315T)23Jlel 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           AX12

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, hemizygous mice may be bred to wild type from the colony. These mice only survive up to 75 days and exhibit progressive neurogegeneration exhibited by decreased motor function.
Mating SystemNoncarrier x Hemizygote         (Female x Male)   03-FEB-12
Hemizygote x Noncarrier         (Female x Male)   03-FEB-12
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 MaleHemizygous for Tg(Prnp-TARDBP*A315T)23Jlel  
Price per Pair (US dollars $)Pair Genotype
$304.00Hemizygous for Tg(Prnp-TARDBP*A315T)23Jlel x Noncarrier  
$304.00Noncarrier x Hemizygous for Tg(Prnp-TARDBP*A315T)23Jlel  

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.

Cryopreserved

Frozen Products

Price (US dollars $)
Frozen Embryo $1650.00

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.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
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*A315T)23Jlel  
Price per Pair (US dollars $)Pair Genotype
$395.20Hemizygous for Tg(Prnp-TARDBP*A315T)23Jlel x Noncarrier  
$395.20Noncarrier x Hemizygous for Tg(Prnp-TARDBP*A315T)23Jlel  

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.

Cryopreserved

Frozen Products

Price (US dollars $)
Frozen Embryo $2145.00

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.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
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
   Noncarrier
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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