Description

Strain Information

Former Names B6.Cg-enrTg(MpbReg)36Pop/J    (Changed: 23-FEB-06 ) B6.Cg-TgN(MbpReg)36Pop enrTgn36Pop    (Changed: 15-DEC-04 ) B6.Cg-enrTgMpbReg)36Pop    (Changed: 15-DEC-04 ) Type Congenic; Mutant Strain; Transgenic; Additional information on Genetically Engineered Mutant Mice. Species laboratory mouse Background Strain C57BL/6J Donor Strain (C57BL/6J x DBA/2J) F1

Appearance
black
Related Genotype: a/a

Description
The enervated (enr^{Tg(MbpReg)36Pop}) mutation resulted from random insertion into a gene of unknown function of a transgene comprising approximately 120 tandem copies of a 1.3 kb segment of the upstream regulatory region of the myelin basic protein gene (Mbp), ending 50 bp upstream of the transcription initiation codon (Kelly et al. 1994). Homozygous mutant mice are characterized by a gait abnormality, stiffened and/or splayed hind legs, and a hunched posture with some exhibiting kyphosis of the thoracic spine. These symptoms progress gradually to severe motor dysfunction. Skeletal muscles show necrosis and phagocytosis. Interactions between Schwann cells and axons are also disrupted. Although many peripheral axons are well myelinated, the nerve and nerve roots contain very large bundles of juxtaposed, bare axons, reminiscent of Schwann cell-axon interactions in early development. Nerve regeneration following injury is also impaired in homozyous mutant mice. There are fewer myelinated fibers per cross sectional area and thinner myelin sheaths surrounding regenerating axons in the nerves of homozygous mutant mice compared to wild type mice at 28 d after crush injury to the sciatic nerve. Abnormal Schwann cell-axon interactions remain in enervated mutant mice as evidenced by the relatively frequent ultrastructural finding of unmyelinated large diameter axons in the regenerating nerves.

Control Information

	Control
	Wild-type from the colony
	000664 C57BL/6J
Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Large

005350	B6.CAST(Cg)-Largevls/Pjn
000226	B6C3Fe a/a-Largemyd/J
000300	MYD/Le-Os +/+ Largemyd/J

View Strains carrying other alleles of Large     (3 strains)

Additional Web Information

Congenic Nomenclature

Phenotype

Phenotype Information

View Research Applications

Research Applications

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

Neurobiology Research
Ataxia (Movement) Defects
Myelination Defects

Genes & Alleles

Gene & Allele Information

Allele Symbol		Largeenr-Tg(MpbReg)36Pop
Allele Name		enervated
Allele Type		Transgenic (random, gene disruption)
Common Name(s)		BPFD #36; Tg(Mbp)36Pop; TgN.BPFD #36; enrTg36Pop;
Strain of Origin		(C57BL/6J x DBA/2J)F1
Gene Symbol and Name		Large, like-glycosyltransferase
Chromosome		8
Gene Common Name(s)		BPFD#36; KIAA0609; MDC1D; Mbp-1; Mbp1; enervated; enr; fg; froggy; mKIAA0609; myd; myelin basic protein transgene; myodystrophy;
Molecular Note		A transgene insertion 160kb downstream of the 3' end of the Large gene results in a severe reduction in the expression of Large mRNA. Expression of six other genes in the immediate vicinity of the transgene insertion was unaltered. Furthermore, noncomplementation tests between enr mutant mice and Largemyd mice demonstrated that these mutations are allelic. [MGI Ref ID J:96939]

Genotyping

Genotyping Information

This strain will not have a genotyping protocol or one is not currently available.

Helpful Links

Optimizing PCR Protocols

References

References

Selected Reference(s)

Kelly D; Chancellor K; Milatovich A; Francke U; Suzuki K; Popko B. 1994. Autosomal recessive neuromuscular disorder in a transgenic line of mice. J Neurosci 14(1):198-207. [PubMed: 7506762]  [MGI Ref ID J:16281]

Additional References

Large^{enr-Tg(MpbReg)36Pop} related

Levedakou EN; Chen XJ; Soliven B; Popko B. 2005. Disruption of the mouse Large gene in the enr and myd mutants results in nerve, muscle, and neuromuscular junction defects. Mol Cell Neurosci 28(4):757-69. [PubMed: 15797722]  [MGI Ref ID J:96939]

Rath EM; Kelly D; Bouldin TW; Popko B. 1995. Impaired peripheral nerve regeneration in a mutant strain of mice (Enr) with a Schwann cell defect. J Neurosci 15(11):7226-37. [PubMed: 7472477]  [MGI Ref ID J:29784]

Health & husbandry

Health & Colony Maintenance Information

Colony Maintenance

Breeding & Husbandry	This strain is being backcrossed onto B6 and the mice require genotyping. Heterozygote x heterozygote matings yield only 13% homozygous progeny. Homozygotes are poor breeders.

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Supply Details

Standard Supply

Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.

Supply Notes

Cryorecovery - Standard. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery. Cryorecovery to establish a Dedicated Supply for greater quantities of mice. One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 or 1-207-288-5845. This strain is included in the Mouse Mutant Resource collection.

Control Information

	Control
	Wild-type from the colony
	000664 C57BL/6J
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.

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Terms of Use

Terms of Use

General Terms and Conditions

Contact information

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phone:	207-288-6470
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