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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Strain Information
Former Names C3HeB/FeJLe-a/a-gnd (Changed: 15-DEC-04 ) Type Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Mating System Ovarian Transplant-Cross-Intercross (Female x Male) Species laboratory mouse Appearance
black, ataxic
Related Genotype: a/a gnd/gnd
black, unaffected
Related Genotype: a/a +/? or a/a gnd/+Important Note
This strain is homozygous for the retinal degeneration allele Pde6brd1.Description
Mice homozygous for the generalized neuroaxonal dystrophy spontanteous mutation (gnd) have large numbers of dystrophic axons in all white matter funiculi and in central grey matter at all levels of the spinal cord. Dystrophic axons also common throughout the brain stem. In the forebrain, some can be seen in the optic nerves and tracts, corpus callosum, rostral commissure, and fornix. Mutant mice are identifiable between 2 and 3 weeks of age by their small size, dull fur, and nervous behavior. Adults are smaller than littermates and have a humped back and slender torso. They walk with a shaky gait and restricted hip movement. Hindlimbs paralyzed by 8 months of age. They rarely breed.
Control Information
| Control | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying Pde6brd1 allele
View Strains carrying Pde6brd1 (74 strains)
004297 B6.CXB1-Pde6brd10/J 002802 C3.BLiA Pde6b+-Krd/J 001979 C3A.BLiA-Pde6b+.O20-Prph2Rd2/J 001912 C3A.BLiA-Pde6b+/J 003648 C3Sn.BLiA-Pde6b+/Dn 004766 C57BL/6J-Pde6brd1-2J/J 004828 FVB.129P2-Pde6b+ Tyrc-ch/AntJ 004808 STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J
Phenotype
Phenotype Information
Neuroaxonal Dystrophy, Infantile; INAD1 - Models with phenotypic similarity to human disease where etiologies are distinct.2
2 Human genes are associated with this disease. Orthologs of those genes do not appear in the mouse genotype(s).
assigned by genotype
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
gnd/gnd
C3H/HeJ-gnd
- growth/size phenotype
- decreased body size (MGI Ref ID J:11718)
- smaller size is identifiable at 2.5 weeks of age
- nervous system phenotype
- axonal dystrophy (MGI Ref ID J:11718)
- dystrophic axons are present in large numbers at all levels of the spinal cord in all white matter funiculi and in central grey matter and are commonly found throughout the brain stem, including nucleus gracilis, cerebellar peduncles and deep cerebellar nuclei, red nucleus, and substantia nigra and in the optic nerve and tract, corpus callosum, rostral commissure and fornix of the forebrain
- behavior/neurological phenotype
- abnormal gait (MGI Ref ID J:11718)
- progressively develop a shaky gait and tend to move the distal hind limbs only below the hock
- at 3 months of age, the hip joint exhibits little flexion and forelimbs tend to splay out to the side
- abnormal motor coordination/ balance (MGI Ref ID J:11718)
- reduced ability to stand on either fore or hind limbs when tested for motor deficits
- abnormal sexual interaction (MGI Ref ID J:11718)
- only rarely do mutants breed
- hindlimb paralysis (MGI Ref ID J:11718)
- hind limbs are paralyzed by 8 months of age and are dragged along behind, however never exhibit spasticity
- hunched posture (MGI Ref ID J:11718)
- at 3 months of age, have a humped back
- skin/coat/nails phenotype
- abnormal coat appearance (MGI Ref ID J:11718)
- exhibit a dull hair coat by 2.5 weeks of age
This mouse can be used to support research in many areas including:Pde6brd1 related
gnd relatedMouse/Human Gene Homologs
retinitis pigmentosa, autosomal recessive
Sensorineural Research
Retinal Degeneration
Neurobiology Research
Ataxia (Movement) Defects
Neurodegeneration
Genes & Alleles
Gene & Allele Information
| Allele Symbol | a | ||
|---|---|---|---|
| Allele Name | nonagouti | ||
| Allele Type | Spontaneous | ||
| Allele Symbol | gnd | ||
| Allele Name | generalized neuroaxonal dystrophy | ||
| Allele Type | Spontaneous | ||
| Strain of Origin | C3H/HeJ | ||
| Gene Symbol and Name | gnd, generalized neuroaxonal dystrophy | ||
| Chromosome | UN | ||
| Allele Symbol | Pde6brd1 | ||
| Allele Name | retinal degeneration 1 | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | Pdebrd1; rd; rd-1; rd1; rodless retina; | ||
Genotyping
Genotyping Information
This strain will not have a genotyping protocol or one is not currently available.
Helpful Links
Optimizing PCR Protocols
References
References
Additional References
Bronson RT; Sweet HO; Spencer CA; Davisson MT. 1992. Genetic and age related models of neurodegeneration in mice: dystrophic axons. J Neurogenet 8(2):71-83. [PubMed: 1634998] [MGI Ref ID J:11718]
gnd relatedBronson RT; Sweet HO; Spencer CA; Davisson MT. 1992. Genetic and age related models of neurodegeneration in mice: dystrophic axons. J Neurogenet 8(2):71-83. [PubMed: 1634998] [MGI Ref ID J:11718]
Health & husbandry
Health & Colony Maintenance Information
Colony Maintenance
Mating System Ovarian Transplant-Cross-Intercross (Female x Male)
Purchasing information
Pricing, Supply Level & Notes, Controls, General Terms & Conditions
Pricing
| Pricing for USA, Canada and Mexico shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
| Pricing for International shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
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Supply Details
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
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| Supply Notes |
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| Important Note | This strain is homozygous for the retinal degeneration allele Pde6brd1. |
Control Information
| Control | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| International - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
General Terms and Conditions
See Terms of Use
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.Ordering and Purchasing Information
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Contact Information
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Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
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Terms of Use
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Contact information
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| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
JAX® Mice & Services Conditions of Use
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