Overview

Also Known As:NMF339, dystonia musculorum(37J)

These mice carry an ENU-induced allelic mutation of the Dst (Dystonin) gene resulting in ataxia.

The Jackson Laboratory cannot guarantee that cryorecovery of strains from the discontinued NIH-funded Neuroscience Mutagenesis Facility (NMF) will be successful or that the anticipated phenotype or genotype will be obtained. The cryorecovery fee for this effort will not be refunded or prorated if the recovery is unsuccessful or is in any way unsatisfactory. Genotyping will be the responsibility of the Purchaser.

Genetic overview

Genetic Background	Generation

Dst^dt-37J

Allele Type	Gene Symbol	Gene Name
Chemically induced (ENU)	Dst	dystonin

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Research Applications

Neurobiology Research

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Detailed Description

The mutants are small and their movement ability is severely limited. They move forward mainly by 'robbing' on their belly, since their front limbs are usually bent at the elbow, and their hind limbs lag behind. No muscle tonus is apparent in either front- or hind limbs upon gently pulling a limb, indicating flaccid paralysis of these limbs. A righting reflex is present only in rudimentary form, i.e. it may take a few seconds before the animal can righten itself from a back-lying position; when picked up by their tail, front- and hind limbs come together, the former are pulled backward, the latter move forward.

Because of phenotypic similarity to dystonia musculorum, complementation tests with Dst^dt-J/J (JR# 0211) have been performed to determine if NMF339 represents an allele of Dst (Dystonin). Four heterozygote matings (NMF339 x Dst^dt-J/J, n=2; Dst^dt-J/J) x NMF339, n=2) produced 8 mutants in a total of 30 progeny, suggesting that NMF339 indeed represents a new allele of Dst^dt-J.

Standard pathology work-up performed on three mutants at 19 (n=1) or 21 (n=2) days of age showed central chromatolysis in neurons of the brain stem and spinal cord, and dystrophic axons. Myelin degeneration, characterized by myelin figures in the cytoplasm of neurons was noted in the peripheral nerves; small muscle fibers and hypoplastic bone marrow (at 21 days of age) were also observed.

These mice are affected at a young age (average onset 2.8 weeks +/- 1; n=21), and the colony had to be maintained through ovarian transplants.

Development

This phenotypic deviant was generated by ethylnitrosourea (ENU) mutagenesis in C57BL/6J males (Stock No. 000664), in the Neuroscience Mutagenesis facility at The Jackson Laboratory. Mutagenized males were crossed to C57BL/6J females; G3 descendants of the mutagenized males were selected for neurological impairment.

Selected References

JAX Neuroscience Mutagenesis Facility. 2003. Heritable mouse mutants from JAX NMF ENU Mutagenesis Program MGI Direct Data SubmissionMGI: J:82238

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Genetics

Dst^dt-37J

Allele Symbol: Dst^dt-37J

Allele Name	dystonia musculorum 37, Jackson
Allele Type	Chemically induced (ENU)
Allele Synonym(s)	Dst^nmf339; neuroscience mutagenesis facility, 339; NMF339
Gene Symbol and Name	Dst, dystonin
Gene Synonym(s)
Strain of Origin	C57BL/6J
Chromosome	1
Molecular Note	This mutation was discovered in an ENU mutagenesis screen and was shown to be an allele of Dst by its failure to complement the dystonia musculorum Jackson mutation.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Neurobiology Research
- Neuroscience Mutagenesis Facility Strain

Genotype: Dst^dt-37J related

Neurobiology Research
- Neurodegeneration
- Neuroscience Mutagenesis Facility Strain

Mammalian Phenotype Terms by Genotype

The following phenotype relates to a compound genotype created using this strain

Genotype: Dst^dt-37J/Dst^dt-37J
C57BL/6J-Dst/J

behavior/neurological phenotype

dystonia
- front limbs remain flexed at the elbow and hind legs drag behind
- locomotion is achieved by the mouse's rocking on its belly
- mutant mice exhibit flaccid paralysis of the fore- and hindlimbs, with an average age of onset of 2.8 weeks
- no muscle tonus is evident when a limb is gently pulled
- when a mutant mouse is picked up by the tail, the front and rear legs come together, the forelimbs dropping backward and the hindlimbs pulled forward

impaired righting response
- mutant mice take a few seconds to right themselves from a back-lying position

nervous system phenotype

axonal dystrophy
- axons of brainstem and spinal cord neurons of 18-21 day old mutants are dystrophic

chromatolysis
- neurons of the brainstem and spinal cord of 18-21 day old mutant mice exhibit central chromatolysis

demyelination
- degeneration of the myelin of peripheral nerves of 18-21 day old mutants is evidenced by myelin figures present in their cytoplasm

growth/size/body region phenotype

decreased body size

hematopoietic system phenotype

decreased bone marrow cell number
- bone marrow of 21-day-old mutants is hypoplastic

muscle phenotype

decreased skeletal muscle fiber size
- the size of muscle fibers is reduced in 21-day old mutant mice

dystonia
- front limbs remain flexed at the elbow and hind legs drag behind
- locomotion is achieved by the mouse's rocking on its belly
- mutant mice exhibit flaccid paralysis of the fore- and hindlimbs, with an average age of onset of 2.8 weeks
- no muscle tonus is evident when a limb is gently pulled
- when a mutant mouse is picked up by the tail, the front and rear legs come together, the forelimbs dropping backward and the hindlimbs pulled forward

References

JAX Neuroscience Mutagenesis Facility. 2003. Heritable mouse mutants from JAX NMF ENU Mutagenesis Program MGI Direct Data SubmissionMGI: J:82238

Additional - Dst^dt-37J related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting
Citation
When using the dystonia musculorum(37J) mouse strain in a publication, please cite the originating article(s) and include JAX stock #005560 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
	Unknown for Dst<dt-37J>

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.

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. 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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General Terms and Conditions

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Licensing Information

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Also Known As:NMF339, dystonia musculorum(37J)

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Selected References

Dstdt-37J

Allele Symbol: Dstdt-37J

Disease Terms

Research Areas By Phenotype

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

Genotype: Dstdt-37J related

Mammalian Phenotype Terms by Genotype

Genotype: Dstdt-37J/Dstdt-37JC57BL/6J-Dst/J

behavior/neurological phenotype

nervous system phenotype

growth/size/body region phenotype

hematopoietic system phenotype

muscle phenotype

References

Additional - Dstdt-37J related

Genotyping Protocols

Citation

Animal Health Reports

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

Live Mouse

Cryorecovery - Pricing

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms Of Use

Licensing Information

JAX® Mice, Products & Services Conditions of Use

No Warranty

Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Dst^dt-37J

Allele Symbol: Dst^dt-37J

Genotype: Dst^dt-37J related

Genotype: Dst^dt-37J/Dst^dt-37J
C57BL/6J-Dst/J

Additional - Dst^dt-37J related