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Former Names C57BL/6J-Clcn1nmf355/J (Changed: 22-MAY-06 ) C57BL/6J-nmf355/J (Changed: 05-JAN-06 ) Type Chemically Induced Mutation; Coisogenic; Mutant Strain; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse H2 Haplotype b Generation N2F2+N1p (04-SEP-05)
Generation DefinitionsAppearance
black, affected
Related Genotype: a/a, Clcn1adr-mto7J/Clcn1adr-mto7J
black, unaffected
Related Genotype: a/a, Clcn1adr-mto7J/+ or a/a, +/+Description
The hind limbs of these mutants show spasms that may be mild or intense; the former result in movement that appears to be 'hesitant', the latter bring the leg close to the body, extend it backwards, or to the side. These spasms affect usually one or the other leg, can last 2 sec or longer, and afterwards the mice move around easily. Lifting the mice by their tail frequently brings the hind limbs close to their body; the withdrawal reflex is impaired or absent, i.e. gently pulling a hind limb does not evoke the normal attempt to withdraw it. The phenotype becomes visible at wean (average onset 4 weeks +/- 0.56 of age, n=30) and both, male or female mice have been affected. The overt phenotype as well as the general mapping results suggested that nmf355 might represent an allele of Clcn1; complementation tests confirmed this, i.e. heterozygote matings with Clcn1adr-mto2J (Jax # 001265) resulted in 3 mutants in a total of 11 progeny, and demonstrated that nmf355 indeed represents an allele of Clcn1. Standard pathology performed on two mutants (33 or 74 days of age) showed no abnormalities. Additional pathology is in progress. The colony is 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.
| Control | ||
|---|---|---|
| +/? untyped from the colony | ||
| Considerations for Choosing Controls | ||
Strains carrying other alleles of Clcn1
003817 B6;129S-Fcgr2btm1Ttk Clcn1adr-mto5J/J 003922 BALB/cByJ-Clcn1adr-mto2J jgl/GrsrJ 005253 C57BL/6J-Clcn1adr-mto6J/J 001265 CByJ.Cg-Clcn1adr-mto2J/J 000939 SWR/J-Clcn1adr-mto/J View Strains carrying other alleles of Clcn1 (5 strains)
View Related Disease (OMIM) Terms
Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested. Myotonia Congenita, Autosomal Dominant (CLCN1)
Myotonia Congenita, Autosomal Recessive (CLCN1)
View Mammalian Phenotype Terms
Mammalian Phenotype Terms provided by MGI
assigned by genotype
Clcn1adr-mto7J/Clcn1adr-mto7J
C57BL/6J-Clcn1adr-mto7J/J
- behavior/neurological phenotype
- limb grasping
- from approximately 4 weeks of age, mutant mice draw their hind limbs close to the body when picked up by the tail and fail to withdraw a hind limb that is gently pulled (MGI Ref ID J:82238)
- muscle phenotype
- muscle spasm
- from approximately 4 weeks of age, mutant mice exhibit hind limb spasms; mild spasms cause apparently hesitant movement, while intense spasms can persist longer than 2 seconds and cause the limb either to be drawn close to the body or extended backward or to the side (MGI Ref ID J:82238)
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:Clcn1adr-mto7J related
Cell Biology Research
Channel and Transporter Defects
chloride
Neurobiology Research
Ataxia (Movement) Defects
Channel and Transporter Defects
chloride
Neuroscience Mutagenesis Facility Strain
| Allele Symbol | Clcn1adr-mto7J | ||
|---|---|---|---|
| Allele Name | myotonia 7 Jackson | ||
| Allele Type | Chemically induced (ENU) | ||
| Common Name(s) | Clcn1nmf355; NMF355; neuroscience mutagenesis facility, 355; | ||
| Strain of Origin | C57BL/6J | ||
| Gene Symbol and Name | Clcn1, chloride channel 1 | ||
| Chromosome | 6 | ||
| Gene Common Name(s) | CLC1; Clc-1; Clc1; NMF355; SMCC; SMCC1; adr; arrested development of righting response; mto; myotonia; neuroscience mutagenesis facility, 355; nmf310; nmf355; | ||
| Molecular Note | This phenotypic allele was identified in an ENU mutagenesis screen. It was shown by its failure to complement Clcn1adr-mto2J to be an allele of Clcn1. | ||
Clcn1adr-mto7J relatedJAX Neuroscience Mutagenesis Facility. 2003. Heritable mouse mutants from JAX NMF ENU Mutagenesis Program MGI Direct Data Submission :. [MGI Ref ID J:82238]
Animal Health Reports
Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.
| Pricing for USA, Canada and Mexico shipping destinations |
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Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2250.00 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.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
- Cryorecovery - Strains Requiring Specialized Phenotyping.
At least two untested males and two untested females (two pairs) will be recovered from cryopreserved stocks and will require specialized phenotyping in order to identify animals carrying the mutation of interest. We do not perform the phenotyping for this strain. Upon completion of the cryorecovery process, we will ship you all of the recovered mice (a minimum of two pair, typically eight or more). We cannot guarantee that you will receive or be able to identify animals of the phenotype of interest. Please be advised that a new order and cryorecovery fee will be necessary to recover additional mice, if needed. The typical cryorecovery period required is 10 weeks. However, because recovery efficiency and viability can vary, a second cryorecovery sometimes may be required in order to complete this order, which may extend the time for order completion to 15 to 20 weeks. If this situation occurs, we will notify you of the delay. Please note that identified 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. Please contact Customer Service for more information: Tel: 1-800-422-6423 (from U.S.A., Canada and Puerto Rico only) or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.
| Pricing for International shipping destinations |
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Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2925.00 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.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
- Cryorecovery - Strains Requiring Specialized Phenotyping.
At least two untested males and two untested females (two pairs) will be recovered from cryopreserved stocks and will require specialized phenotyping in order to identify animals carrying the mutation of interest. We do not perform the phenotyping for this strain. Upon completion of the cryorecovery process, we will ship you all of the recovered mice (a minimum of two pair, typically eight or more). We cannot guarantee that you will receive or be able to identify animals of the phenotype of interest. Please be advised that a new order and cryorecovery fee will be necessary to recover additional mice, if needed. The typical cryorecovery period required is 10 weeks. However, because recovery efficiency and viability can vary, a second cryorecovery sometimes may be required in order to complete this order, which may extend the time for order completion to 15 to 20 weeks. If this situation occurs, we will notify you of the delay. Please note that identified 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. Please contact Customer Service for more information: Tel: 1-800-422-6423 (from U.S.A., Canada and Puerto Rico only) or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.
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Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
| Control | ||
|---|---|---|
| +/? untyped from the colony | ||
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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