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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
- Disease & phenotype
- Genes & alleles
- Genotyping
- Health & care
- References
- Pricing & purchasing
- Terms of use
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 System Hemizygote x Hemizygote (Female x Male) 17-OCT-11 Species laboratory mouse Generation F?+F3 (21-DEC-12)
Generation DefinitionsDonating 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
Amyotrophic Lateral Sclerosis (ALS)
009680 B6.B-Vps54wr/J 010700 B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J 002298 B6.Cg-Tg(SOD1)2Gur/J 016149 B6.Cg-Tg(SOD1*G37R)1Dwc/J 008229 B6.Cg-Tg(SOD1*G37R)29Dpr/J 008342 B6.Cg-Tg(SOD1*G37R)42Dpr/J 008248 B6.Cg-Tg(SOD1*G85R)148Dwc/J 004435 B6.Cg-Tg(SOD1*G93A)1Gur/J 002299 B6.Cg-Tg(SOD1*G93A)dl1Gur/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 002297 B6SJL-Tg(SOD1)2Gur/J 002726 B6SJL-Tg(SOD1*G93A)1Gur/J 002300 B6SJL-Tg(SOD1*G93A)dl1Gur/J 017604 C57BL/6-Tg(Prnp-TARDBP*M337V)4Ptrc/J 002628 C57BL/6-Tg(SOD1)10Cje/J 002629 C57BL/6-Tg(SOD1)3Cje/J 005706 C57BL/6-Tg(tetO-CDK5R1/GFP)337Lht/J 008230 FVB(Cg)-Tg(Thy1-SOD1*G93A)T3Hgrd/J 005110 FVB-Tg(Sod1*G86R)M1Jwg/J 013199 FVB.Cg-Tg(SOD1*G93A)1Gur/J 013574 FVB/N-Tg(149m19)M141Kunst/J 017916 STOCK Tg(Prnp-FUS)WT3Cshw/J 016144 STOCK Tg(Prnp-TARDBP)4Jlel/J 016143 STOCK Tg(Prnp-TARDBP*A315T)23Jlel/J View Amyotrophic Lateral Sclerosis (ALS) (27 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
- 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
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 phenotype
- decreased body weight
- body weight is reduced from 14 days of age onward (MGI Ref ID J:163231)
- behavior/neurological phenotype
- abnormal gait
- 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
- 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 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)
This mouse can be used to support research in many areas including:
Mouse/Human Gene Homologs
amyotrophic lateral sclerosis (ALS)
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 Origin | C57BL/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), High Resolution Melting
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]
Health & husbandry
Health & Colony Maintenance Information
Animal Health Reports
Room Number FGB29
Colony Maintenance
Breeding & Husbandry When maintaining a live colony, these mice may be bred as hemizygotes. Mating System Hemizygote 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 |
|
Live Mice
Price per mouse (US dollars $) Gender Genotypes Provided Individual Mouse $232.00 Female or Male Hemizygous for Tg(Prnp-TARDBP)3cPtrc $232.00 Female or Male Homozygous for Tg(Prnp-TARDBP)3cPtrc
Price per Pair (US dollars $) Pair Genotype $464.00 Hemizygous for Tg(Prnp-TARDBP)3cPtrc x Hemizygous for Tg(Prnp-TARDBP)3cPtrc $296.00 Hemizygous for Tg(Prnp-TARDBP)3cPtrc x Noncarrier $296.00 Noncarrier x Hemizygous for Tg(Prnp-TARDBP)3cPtrc Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order 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.
| Pricing for International shipping destinations |
|
Live Mice
Price per mouse (US dollars $) Gender Genotypes Provided Individual Mouse $301.60 Female or Male Hemizygous for Tg(Prnp-TARDBP)3cPtrc $301.60 Female or Male Homozygous for Tg(Prnp-TARDBP)3cPtrc
Price per Pair (US dollars $) Pair Genotype $603.20 Hemizygous for Tg(Prnp-TARDBP)3cPtrc x Hemizygous for Tg(Prnp-TARDBP)3cPtrc $384.80 Hemizygous for Tg(Prnp-TARDBP)3cPtrc x Noncarrier $384.80 Noncarrier x Hemizygous for Tg(Prnp-TARDBP)3cPtrc Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order 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.
|
|
|
Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order 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.
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 000664 C57BL/6J | (approximate) | |
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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