Strain Name:

B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J

Stock Number:

010700

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Availability:

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Use Restrictions Apply, see Terms of Use
Prp-TDP43A315T transgenic mice express a mutant human TAR DNA binding protein cDNA harboring an amino acid substitution associated with familial ALS. Hemizygous mice develop a progressive and fatal neurodegenerative disease reminiscent of both ALS and frontotemporal lobar degeneration with ubiquitin aggregates. 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 B6;CB-Tg(Prnp-TARDBP*A315T)95Balo/J    (Changed: 23-MAR-11 )
B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J    (Changed: 22-DEC-09 )
Type Congenic; Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Mating SystemNoncarrier x Hemizygote         (Female x Male)   26-MAR-10
Mating SystemHemizygote x Noncarrier         (Female x Male)   06-FEB-12
Specieslaboratory mouse
GenerationN5+N11F1 (11-DEC-13)
Generation Definitions
 
Donating Investigator Robert H Baloh,   Washington University School of Medicine

Description
Mice hemizygous for this Prp-TDP43A315T transgene are viable, fertile, and express a mutant human TAR DNA binding protein (TARDBP or TDP-43) cDNA harboring an N-terminal Flag tag and an A315T amino acid substitution that is associated with familial Amyotrophic Lateral Sclerosis (ALS). Expression is directed throughout the nervous system by mouse prion protein (PrP or Prnp) promoter/enhancer regions.

Hemizygous mice were originally published on a mixed C57BL/6;CBA genetic background and develop a progressive gait disorder around 3-4 months of age with death around 5 months of age. For hemizygous mice on a mixed C57BL/6;CBA genetic background, the donating investigator reports that, on average, males die almost one month earlier than females. Due to continued backcrossing to C57BL/6J at The Jackson Laboratory Repository, this strain is now fully congenic on a C57BL/6J background. Survival differences between male and female hemizygous mice are still observed. However, hemizygous males on a C57BL/6J genetic background have an average survival time of approximately 3.5 months (97 +/- 11 days); this is earlier lethality than hemizygous males on a mixed C57BL/6;CBA genetic background. On a C57BL/6J genetic background, hemizygous females live significantly longer than hemizygous males. In addition, male mice exhibit a progressive neurodegeneration in the myenteric plexus of the colon, which is characterized by reduced GI motility and contributes to the decreased lifespan observed in these mice. This phenotype is most severe in males on the C57BL/6J background.For more detailed information, please view graph of B6.Tg(Prnp-TARDBP*A315T)95Balo/J survival and data 2010-2012. [pdf] The intestinal dysmotility phenotype observed in these mice can be mitigated by the use of jellified food. Mice fed jellifed food survive and develop a progressive motor phenotype including gait abnormalities, denervated neuromuscular junctions, gastrocnemius muscle atrophy, and upper and lower motor axon loss (especially large axons). (Herdewyn et. al. Mol Neuro 9:24, 2014)

The progressive and fatal neurodegenerative disease phenotype of hemizygous mice is reminiscent of both ALS and frontotemporal lobar degeneration with ubiquitin aggregates (FTLD-U). Specifically, hemizygous mice accumulate pathologic aggregates of ubiquitinated proteins only in specific neuronal populations, including frontal cortex layer V pyramidal neurons and spinal motor neurons with activation of local astrocytes and microglia. Loss of both upper and lower motor neurons is also observed. TDP-43 aggregates are not reported in the cytoplasm. The donating investigator has not attempted to make homozygous mice.

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. As the Prp-TDP43A315T transgenic mice were originally created on a mixed C57BL/6;CBA genetic background, it should be noted that the phenotype of these Prp-TDP43A315T transgenic mice on a C57BL/6-congenic background may vary greatly 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 modified to harbor an N-terminal Flag tag immediately after the start methionine and the A315T amino acid substitution associated with familial ALS. This Flag-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 oocytes from hybrid C57BL/6J x CBA mice and a single founder mouse was bred with C57BL/6 wildtype mice to establish Prp-TDP43A315T founder line 95. These mice were then backcrossed to C57BL/6J inbred mice for approximately five generations (with black or agouti coat color) prior to sending agouti mice to The Jackson Laboratory Repository. Upon arrival, transgenic mice were bred with C57BL/6J inbred mice (Stock No. 000664) to establish the colony. A subsequent genome scan revealed this stock to be approximately 80% congenic to C57BL/6J. After this, hemizygous mice were additionally backcrossed to C57BL/6J inbred mice for multiple generations to make the mice fully congenic on the C57BL/6J genetic background.

Control Information

  Control
   Noncarrier
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

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003378   B6C3-Tg(APP695)3Dbo Tg(PSEN1)5Dbo/J
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008083   B6CBA-Tg(Prnp-TBP*)13Xjl/J
003741   B6D2-Tg(Prnp-MAPT)43Vle/J
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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
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
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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     (39 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 Dementia; FTD
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Tg(Prnp-TARDBP*A315T)95Balo/0

        B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J
  • behavior/neurological phenotype
  • hypoactivity
    • late onset immobility occurs suddenly and progresses rapidly especially in males   (MGI Ref ID J:203041)
  • impaired coordination
    • time-to-fall as measured by the hanging wire test is significantly decreased in transgenic males as compared to wild type littermates   (MGI Ref ID J:203041)
  • impaired righting response
    • late onset difficulty in righting occurs suddenly and progresses rapidly especially in males   (MGI Ref ID J:203041)
  • limb grasping
    • mild deficit in the hindlimb reflex is observed in both male and female mice   (MGI Ref ID J:203041)
  • tail dragging
    • both male and female mice exhibit a tendency to drag their tails when walking   (MGI Ref ID J:203041)
  • digestive/alimentary phenotype
  • abnormal intestinal transit time
    • gut motility is significantly decreased beginning at day 60 in males and progresses to 282% of controls by day 90   (MGI Ref ID J:203041)
    • gut motility is significantly decreased in females but at later age than males and with less severity   (MGI Ref ID J:203041)
  • abnormal intestine physiology
    • lower GI tract develops swelling, intra-intestinal coagulated blood, and necrotic tissue, however, pathology of upper digestive tract is normal   (MGI Ref ID J:203041)
    • intestinal edema   (MGI Ref ID J:203041)
  • decreased defecation amount
    • progressive decrease in number of fecal pellets excreted   (MGI Ref ID J:203041)
  • growth/size/body phenotype
  • distended abdomen
    • late onset swollen and tender abdomen occurs suddenly and progresses rapidly especially in males   (MGI Ref ID J:203041)
  • weight loss
    • weight loss becomes significant at post-natal day 85 in males and post-natal day 127 in females   (MGI Ref ID J:203041)
  • homeostasis/metabolism phenotype
  • dehydration
    • late onset dehydration occurs suddenly and progresses rapidly especially in males   (MGI Ref ID J:203041)
  • intestinal edema   (MGI Ref ID J:203041)
  • integument phenotype
  • disheveled coat
    • late onset unkempt haircoat occurs suddenly and progresses rapidly especially in males   (MGI Ref ID J:203041)
  • mortality/aging
  • abnormal induced morbidity/mortality
    • ovariectomy decreases female median survival by 94 days   (MGI Ref ID J:203041)
    • male castration decreases median survival by 10 days   (MGI Ref ID J:203041)
  • premature death
    • death occurs at a median of 108 days in males death occurs at a median of 108 days in males   (MGI Ref ID J:203041)
    • death occurs at a median of 185 days in females death occurs at a median of 185 days in females   (MGI Ref ID J:203041)
  • nervous system phenotype
  • abnormal axon morphology
    • small, but significant decrease in axon number and diameter in the sensory branch of the femoral nerve in 3 and 5 month old males   (MGI Ref ID J:203041)
  • abnormal enteric ganglia morphology
    • ganglions in the superior mesentery appear smaller than control ganglions   (MGI Ref ID J:203041)
    • ganglions exhibit an increased percentage of condensed nuclei   (MGI Ref ID J:203041)
  • abnormal motor neuron morphology
    • the large motor neurons of some males exhibit abnormally shaped nuclei   (MGI Ref ID J:203041)
    • motor neuron degeneration   (MGI Ref ID J:203041)
  • abnormal myenteric nerve plexus morphology
    • 80% decrease in the number of neurons per ganglion in the myenteric plexus of the colon, but not the duodenum, of 90-150 day old mice   (MGI Ref ID J:203041)
  • astrocytosis
    • reactive astrocytosis observed in spinal cord lumbar sections from 80 day old males   (MGI Ref ID J:203041)
  • deficient enteric cholinergic innervation
    • decreased AChE staining in the ganglion and intermodal strands of the myenteric plexus of the colon   (MGI Ref ID J:203041)
  • neuronal cytoplasmic inclusions
    • some motor neurons have ubiquitin-positive cytoplasmic inclusions   (MGI Ref ID J:203041)
  • skeleton phenotype
  • kyphosis
    • late onset kyphosis occurs suddenly and progresses rapidly especially in males   (MGI Ref ID J:203041)

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

Tg(Prnp-TARDBP*A315T)95Balo/0

        involves: C57BL/6 * CBA
  • mortality/aging
  • premature death
    • average survival is 154 days; at end-stage, mice die spontaneously or are euthanized when they lose righting reflex or can no longer obtain food and water   (MGI Ref ID J:153197)
  • growth/size/body phenotype
  • weight loss
    • mice begin losing weight around 4.5 months of age   (MGI Ref ID J:153197)
  • behavior/neurological phenotype
  • abnormal gait
    • at 3-4 months mice develop abnormal gait; around 4.5 months, mice exhibit a "swimming" gait when they lose ability to support their body weight but still use their limbs for propulsion to slide on their stomachs   (MGI Ref ID J:153197)
  • nervous system phenotype
  • abnormal neuron morphology
    • late-stage mice have cytoplasmic accumulation of ubiquinated proteins in neurons of cortical layer 5; these neurons are prominent in the motor cortex and are also present in orbital, cingulated, sensory and other cortical regions   (MGI Ref ID J:153197)
    • no ubiquinated protein aggregates are observed in the caudate/putamen, substantia nigra, thalamus or other structures at any stage   (MGI Ref ID J:153197)
    • decreased motor neuron number
      • in end-stage mice, about a 20% loss of spinal motor neurons is observed   (MGI Ref ID J:153197)
    • neuron degeneration
      • neuron loss in cortical layer 5   (MGI Ref ID J:153197)
      • motor neuron degeneration
        • fewer axons are observed in the lower thoracic spinal cord with numerous degenerating axons being seen in dorsal corticospinal tract and lateral columns   (MGI Ref ID J:153197)
        • femoral motor and sensory nerves shows loss of axons with ongoing axonal degeneration in the motor branch   (MGI Ref ID J:153197)
  • abnormal spinal cord morphology
    • fewer axons are observed in the lower thoracic spinal cord with numerous degenerating axons being seen in dorsal corticospinal tract and lateral columns   (MGI Ref ID J:153197)
    • abnormal spinal cord ventral horn morphology
      • presence of ubiquitinated protein accumulations is detected preferentially in large neurons of the dorsal horn as well as scattered interneurons   (MGI Ref ID J:153197)
    • decreased motor neuron number
      • in end-stage mice, about a 20% loss of spinal motor neurons is observed   (MGI Ref ID J:153197)
  • astrocytosis
    • activation of astrocytes is detected in layer 5 with reactive astrocytosis seen around degenerating neurons   (MGI Ref ID J:153197)
  • muscle phenotype
  • abnormal muscle electrophysiology
    • electromyography in end-stage mice shows numerous fibrillation potentials indicative of loss of muscle fiber innervation and fasciculations, which are spontaneous firing of motor units often seen with human motor neuron diseases; in presymptomatic and early-stage mice, electromyography is normal   (MGI Ref ID J:153197)
  • abnormal skeletal muscle morphology
    • end-stage mice have scattered and grouped atrophic muscle fibers, characteristic of muscle denervation   (MGI Ref ID J:153197)
    • abnormal skeletal muscle fiber morphology
      • atrophic muscle fibers are observed in end-stage mice   (MGI Ref ID J:153197)
  • muscle weakness
    • around 4.5 months, mice can no longer support their body weight   (MGI Ref ID J:153197)

Tg(Prnp-TARDBP*A315T)95Balo/0

        (FVB/NJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
  • mortality/aging
  • extended life span
    • survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background   (MGI Ref ID J:203041)
  • digestive/alimentary phenotype
  • abnormal intestine physiology
    • all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background   (MGI Ref ID J:203041)

Tg(Prnp-TARDBP*A315T)95Balo/0

        (ALR/LtJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
  • mortality/aging
  • extended life span
    • survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background   (MGI Ref ID J:203041)
  • digestive/alimentary phenotype
  • abnormal intestine physiology
    • all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background   (MGI Ref ID J:203041)

Tg(Prnp-TARDBP*A315T)95Balo/0

        (129S1/SvImJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
  • mortality/aging
  • extended life span
    • survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background   (MGI Ref ID J:203041)
  • digestive/alimentary phenotype
  • abnormal intestine physiology
    • all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background   (MGI Ref ID J:203041)

Tg(Prnp-TARDBP*A315T)95Balo/0

        (PWK/PhJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
  • mortality/aging
  • extended life span
    • survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background   (MGI Ref ID J:203041)
  • digestive/alimentary phenotype
  • abnormal intestine physiology
    • all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background   (MGI Ref ID J:203041)

Tg(Prnp-TARDBP*A315T)95Balo/0

        (WSB/EiJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
  • mortality/aging
  • extended life span
    • survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background   (MGI Ref ID J:203041)
  • digestive/alimentary phenotype
  • abnormal intestine physiology
    • all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background   (MGI Ref ID J:203041)

Tg(Prnp-TARDBP*A315T)95Balo/0

        (C3H/HeJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
  • mortality/aging
  • extended life span
    • survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background   (MGI Ref ID J:203041)
  • digestive/alimentary phenotype
  • abnormal intestine physiology
    • all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background   (MGI Ref ID J:203041)
View Research Applications

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

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

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(Prnp-TARDBP*A315T)95Balo
Allele Name transgene insertion 95, Robert Baloh
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) Prp-TDP43-A315T; Prp-TDP43A315T; Prp-TDP43A315T;
Mutation Made By Robert Baloh,   Washington University School of Medicine
Strain of OriginC57BL/6 x CBA
Expressed Gene TARDBP, TAR DNA binding protein, human
Promoter Prnp, prion protein, mouse, laboratory
Molecular Note The construct contains the full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence that was modified to harbor an N-terminal Flag tag immediately after the start methionine and the A315T amino acid substitution associated with familial ALS inserted between exon 2 and exon 3 of mouse prion protein (PrP or Prnp) gene. The Prp-TDP43A315T transgene was microinjected into oocytes from hybrid C57BL/6J x CBA mice and a single Prp-TDP43A315T founder line was established (founder line 95). [MGI Ref ID J:153197]
 
 

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)

Hatzipetros T; Bogdanik LP; Tassinari VR; Kidd JD; Moreno AJ; Davis C; Osborne M; Austin A; Vieira FG; Lutz C; Perrin S. 2013. C57BL/6J congenic Prp-TDP43A315T mice develop progressive neurodegeneration in the myenteric plexus of the colon without exhibiting key features of ALS. Brain Res :. [PubMed: 24141148]  [MGI Ref ID J:203041]

Wegorzewska I; Bell S; Cairns NJ; Miller TM; Baloh RH. 2009. TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A :. [PubMed: 19833869]  [MGI Ref ID J:153197]

Additional References

Tg(Prnp-TARDBP*A315T)95Balo related

Dang TN; Dobson-Stone C; Glaros EN; Kim WS; Hallupp M; Bartley L; Piguet O; Hodges JR; Halliday GM; Double KL; Schofield PR; Crouch PJ; Kwok JB. 2013. Endogenous progesterone levels and frontotemporal dementia: modulation of TDP-43 and Tau levels in vitro and treatment of the A315T TARDBP mouse model. Dis Model Mech 6(5):1198-204. [PubMed: 23798570]  [MGI Ref ID J:201688]

Guo Y; Wang Q; Zhang K; An T; Shi P; Li Z; Duan W; Li C. 2012. HO-1 induction in motor cortex and intestinal dysfunction in TDP-43 A315T transgenic mice. Brain Res 1460:88-95. [PubMed: 22578468]  [MGI Ref ID J:186444]

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]

Herdewyn S; Cirillo C; Van Den Bosch L; Robberecht W; Vanden Berghe P; Van Damme P. 2014. Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice. Mol Neurodegener 9(1):24. [PubMed: 24938805]  [MGI Ref ID J:210295]

Magrane J; Cortez C; Gan WB; Manfredi G. 2014. Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models. Hum Mol Genet 23(6):1413-24. [PubMed: 24154542]  [MGI Ref ID J:206221]

McGoldrick P; Joyce PI; Fisher EM; Greensmith L. 2013. Rodent models of amyotrophic lateral sclerosis. Biochim Biophys Acta 1832(9):1421-36. [PubMed: 23524377]  [MGI Ref ID J:202416]

Medina DX; Orr ME; Oddo S. 2014. Accumulation of C-terminal fragments of transactive response DNA-binding protein 43 leads to synaptic loss and cognitive deficits in human TDP-43 transgenic mice. Neurobiol Aging 35(1):79-87. [PubMed: 23954172]  [MGI Ref ID J:211978]

Perera ND; Sheean RK; Scott JW; Kemp BE; Horne MK; Turner BJ. 2014. Mutant TDP-43 Deregulates AMPK activation by PP2A in ALS models. PLoS One 9(3):e90449. [PubMed: 24595038]  [MGI Ref ID J:214698]

Srivastava A; Philip VM; Greenstein I; Rowe LB; Barter M; Lutz C; Reinholdt LG. 2014. Discovery of transgene insertion sites by high throughput sequencing of mate pair libraries. BMC Genomics 15:367. [PubMed: 24884803]  [MGI Ref ID J:214367]

Wenqiang C; Lonskaya I; Hebron ML; Ibrahim Z; Olszewski RT; Neale JH; Moussa CE. 2014. Parkin-mediated reduction of nuclear and soluble TDP-43 reverses behavioral decline in symptomatic mice. Hum Mol Genet 23(18):4960-9. [PubMed: 24847002]  [MGI Ref ID J:214311]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX10

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, hemizygous carriers may be bred with wildtype (noncarrier) mice from the colony or C57BL/6J inbred mice (Stock No. 000664). Hemizygous males on a C57BL/6J genetic background have an average survival time of approximately 3.5 months (97 +/- 11 days); this is earlier lethality than hemizygous males on a mixed C57BL/6;CBA genetic background. On a C57BL/6J genetic background, hemizygous females live significantly longer than hemizygous males. The intestinal dysmotility phenotype and sudden death observed in these mice can be mitigated by the use of jellified food.(Herdewyn et. al. Mol Neuro 9:24, 2014)The donating investigator has not attempted to make homozygous mice.
Mating SystemNoncarrier x Hemizygote         (Female x Male)   26-MAR-10
Hemizygote x Noncarrier         (Female x Male)   06-FEB-12
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 $239.00Female or MaleHemizygous for Tg(Prnp-TARDBP*A315T)95Balo  
Price per Pair (US dollars $)Pair Genotype
$261.55C57BL/6J (000664) x Hemizygous for Tg(Prnp-TARDBP*A315T)95Balo  
$260.05Hemizygous for Tg(Prnp-TARDBP*A315T)95Balo x C57BL/6J (000664)  

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 $310.70Female or MaleHemizygous for Tg(Prnp-TARDBP*A315T)95Balo  
Price per Pair (US dollars $)Pair Genotype
$340.10C57BL/6J (000664) x Hemizygous for Tg(Prnp-TARDBP*A315T)95Balo  
$338.10Hemizygous for Tg(Prnp-TARDBP*A315T)95Balo x C57BL/6J (000664)  

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

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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JAX® Mice
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Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
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Terms of Use

Terms of Use


General Terms and Conditions


For Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.

Contact information

General inquiries regarding Terms of Use

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phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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