Overview

Also Known As:myelin deficient

These mice carry an autosomal recessive spontaneous mutation that produces deficiencies in central nervous system myelin, and quaking or trembling of the hindquarters during locomotion.

Genetic overview

Genetic Background	Generation

Allele Type	Gene Symbol	Gene Name
Spontaneous	Mbp	myelin basic protein

Research Applications

Sensorineural Research
Developmental Biology Research
Neurobiology Research

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Detailed Description

These mice carry an autosomal recessive spontaneous mutation that produces deficiencies in central nervous system myelin, and quaking or trembling of the hindquarters during locomotion.

Control Suggestions

Wild-type from the colony

Additional Information

Considerations for Choosing Controls

Genetics

Mbp^shi-mld

Allele Symbol: Mbp^shi-mld

Allele Name	myelin deficient
Allele Type	Spontaneous
Allele Synonym(s)
Gene Symbol and Name	Mbp, myelin basic protein
Gene Synonym(s)
Strain of Origin	MDB/Dt
Chromosome	18
General Note	The Mbp^shi-mld mutation arose spontaneously in the MDB/Dt strain at Purdue University (J:5917). Two stocks of Mbp^shi-mld-bearing mice, both derived from the Purdue original stock at about the same time, and maintained respectively in Worcester, Massachusetts, and in Lausanne, Switzerland, show quantitative differences in life span, in incidence of convulsions, in MBP at older ages, and in proportion of optic axons myelinated. The differences are attributed to differences in regulatory genes in the two stocks, maintained on quite different backgrounds (J:27485).The effects of the Mbp^shi-mld mutation are less severe than those of Mbpshi. MBP is present in the CNS, though low in quantity, and the myelin deficient phenotype is rendered normal by an Mbp transgene (J:6320). Mbp^shi/Mbp^shi-mld heterozygotes are deficient in CNS myelin to a greater extent than Mbp^shi-mld/Mbp^shi-mld homozygotes, but less than Mbp^shi/Mbp^shi homozygotes (J:27481). Mbpshi-mld mice have normal lipid/protein ratios, lipid distribution patterns, and only moderate abnormalities of fatty acid profiles (J:27484), whereas these elements are strongly abnormal in Mbpshi (J:6012). Mbpshi caused more severe deficits in learning performance than Mbpshi-mld, while neither affected natural behavior (J:27482).
Molecular Note	This mutation comprises a tandem duplication and inversion of Mbp. Recombination points indicate that the duplication and inversion event was the result of nonhomologous recombination. Antisense RNA produced by the duplicated gene complexes with sense RNA reducing Mpb expression.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Mbp^shi-mld related

Sensorineural Research
- Hearing Defects
Neurobiology Research
- Myelination Defects
- Tremor Defects
- Hearing Defects

Developmental Biology Research
- Postnatal Lethality
  - Homozygous

Mammalian Phenotype Terms by Genotype

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

Genotype: Mbp^shi-mld/Mbp^shi-mld
involves: MDB/Dt

behavior/neurological phenotype

abnormal behavior

tremors
- rapid trembling of hind limbs and caudal trunk during normal movement
- detected in mutant mice at 10-14 days of age
- not observed when mice are at rest

sporadic seizures
- beginning at one month of age
- followed by complete prostration

mortality/aging

premature death
- mutant mice reach sexually maturity

nervous system phenotype

sporadic seizures
- beginning at one month of age
- followed by complete prostration

References

Additional References

Akowitz AA; Barbarese E; Scheld K; Carson JH. 1987. Structure and expression of myelin basic protein gene sequences in the mld mutant mouse: reiteration and rearrangement of the MBP gene. Genetics 116(3):447-64PubMed: 2440764 MGI: J:8798
Chernoff GF. 1981. Shiverer: an autosomal recessive mutant mouse with myelin deficiency. J Hered 72(2):128PubMed: 6168677 MGI: J:6578
Doolittle DP; Schweikart KM. 1977. Myelin deficient, a new neurological mutant in the mouse. J Hered 68(5):331-2PubMed: 599283 MGI: J:5917
Matthieu JM; Ginalski H; Friede RL; Cohen SR; Doolittle DP. 1980. Absence of myelin basic protein and major dense line in CNS myelin of the mld mutant mouse. Brain Res 191(1):278-83PubMed: 6155168 MGI: J:6320
Matthieu JM; Omlin FX; Ginalski-Winkelmann H; Cooper BJ. 1984. Myelination in the CNS of mld mutant mice: comparison between composition and structure. Brain Res 315(1):149-58PubMed: 6202376 MGI: J:26989
Matthieu JM; Tosic M; Roach A. 1992. Myelin-deficient mutant mice. An in vivo model for inhibition of gene expression by natural antisense RNA. Ann N Y Acad Sci 660:188-92PubMed: 1285071 MGI: J:4161
Nakajima K; Ikenaka K; Kagawa T; Aruga J; Nakao J; Nakahira K; Shiota C; Kim SU; Mikoshiba K. 1993. Novel isoforms of mouse myelin basic protein predominantly expressed in embryonic stage. J Neurochem 60(4):1554-63PubMed: 7681106 MGI: J:4643
Roach A; Takahashi N; Pravtcheva D; Ruddle F; Hood L. 1985. Chromosomal mapping of mouse myelin basic protein gene and structure and transcription of the partially deleted gene in shiverer mutant mice. Cell 42(1):149-55PubMed: 2410137 MGI: J:7925

Additional - Mbp^shi-mld related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting
Breeding Considerations

While maintaining a live colony, heterozygous mice may be bred together, to wildtype mice from the colony or to C57BL/6J inbred mice (Stock No. 000664). Homozygous mice die before 2 months of age. These mice are extremely sensitive to noise.
- Additional Breeding and Husbandry Support
Citation
When using the myelin deficient mouse strain in a publication, please cite the originating article(s) and include JAX stock #002492 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

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Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service/Product	Description	Price
> >	Heterozygous for Mbp<shi-mld>

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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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