Overview

Also Known As:generalized neuroaxonal dystrophy

These mice carry the spontaneous gnd mutation and are characterized by dystrophic axons of the nervous system.

Genetic overview

Genetic Background	Generation

a

Allele Type	Gene Symbol	Gene Name
Spontaneous	a	nonagouti

Allele Type	Gene Symbol	Gene Name
Spontaneous	gnd	generalized neuroaxonal dystrophy

Research Applications

Neurobiology Research

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Important Note

This strain is homozygous for the retinal degeneration allele Pde6b^rd1.

Detailed 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.

Development

The generalized neuroaxonal dystrophy mutation (gnd) arose spontaneously on the agouti C3H/HeJ inbred background at The Jackson Laboratory in 1982, when that inbred was at generation F175. This mutation was maintained via homozygous ovarian transplant into nonagouti C3FeLe.B6-a/J then bred to C3FeLe.B6-a/J males. In this way the offspring that derived from any residual host oocytes could be eliminated based on black coat color and the obligate heterozygous agouti offspring were then intercrossed to generate homozygous females for ovarian transplantation. The strain was maintained via this ovarian transplant backcross-intercross method for over a decade. In 1998 sperm was cryopreserved from homozygous and heterozygous males at backcross generation N24.

Control Suggestions

Untyped from the colony

Additional Information

Considerations for Choosing Controls

Genetics

a

Allele Symbol: a

Allele Name	nonagouti
Allele Type	Spontaneous
Allele Synonym(s)
Gene Symbol and Name	a, nonagouti
Gene Synonym(s)
Strain of Origin	old mutant of the mouse fancy
Chromosome	2
General Note	Insertion of the LV30 retrotransposon without the beta4 retrovirus sequence does not cause the nonagouti phenotype. J:278039
Molecular Note	Characterization of this allele shows an insertion of DNA comprised of a 5.5kb virus-like element, VL30, into the first intron of the agouti gene. The VL30 element itself contains an additional 5.5 kb sequence, flanked by 526 bp of direct repeats (beta4 retroviral sequence). The host integration site is the same as for a^t-2Gso and A^w-38J and includes a duplication of four nucleotides of host DNA and a deletion of 2 bp from the end of each repeat. Northern analysis of mRNA from skin of homozygotes shows a smaller agouti message and levels 8 fold lower than found in wild-type.

gnd

Allele Symbol: gnd

Allele Name	generalized neuroaxonal dystrophy
Allele Type	Spontaneous
Allele Synonym(s)
Gene Symbol and Name	gnd, generalized neuroaxonal dystrophy
Gene Synonym(s)
Strain of Origin	C3H/HeJ
Chromosome	UN

Disease/Phenotype

Disease Terms

Models with phenotypic similarity to human diseases where etiology is unknown or involving genes where ortholog is unknown.

neurodegeneration with brain iron accumulation 2a

Research Areas By Phenotype

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

Genotype: gnd related

Neurobiology Research
- Neurodegeneration
- Ataxia (Movement) Defects

Mammalian Phenotype Terms by Genotype

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

Genotype: gnd/gnd
C3H/HeJ-gnd

behavior/neurological phenotype

abnormal gait
- at 3 months of age, the hip joint exhibits little flexion and forelimbs tend to splay out to the side
- progressively develop a shaky gait and tend to move the distal hind limbs only below the hock

abnormal motor coordination/balance
- reduced ability to stand on either fore or hind limbs when tested for motor deficits

abnormal sexual interaction
- only rarely do mutants breed

hindlimb paralysis
- hind limbs are paralyzed by 8 months of age and are dragged along behind, however never exhibit spasticity

hunched posture
- at 3 months of age, have a humped back

growth/size/body region phenotype

decreased body size
- smaller size is identifiable at 2.5 weeks of age

integument phenotype

abnormal coat appearance
- exhibit a dull hair coat by 2.5 weeks of age

nervous system phenotype

axonal dystrophy
- 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
- uclei, red nucleus, and substantia nigra and in the optic nerve and tract, corpus callosum, rostral commissure and fornix of the forebrain

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):71-83PubMed: 1634998 MGI: J:11718

Additional - a related

Additional - gnd related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting
Mating System
- Ovarian Transplant-Cross-Intercross
Appearance
- black, ataxic
  Related Genotype: a/a gnd/gnd
  
  black, unaffected
  Related Genotype: a/a +/? or a/a gnd/+
Citation
When using the generalized neuroaxonal dystrophy mouse strain in a publication, please cite the originating article(s) and include JAX stock #001886 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service/Product	Description	Price
	Progeny testing required but not provided. No genotyping assay is available for these recessive cryo-recovered animals of undefined genotype

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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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

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