Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Former Names C57BL/6J-Nmf134/J    (Changed: 18-JUL-08 ) C57BL/6J-Nmf134    (Changed: 15-DEC-04 ) NMF134    (Changed: 15-DEC-04 ) Type Chemically Induced Mutation; Mutant Strain; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation N3F2p (25-JAN-04)

Appearance
nonagouti black
Related Genotype: a/a

Important Note
The C57BL/6J background strain is homozygous for the age related hearing loss mutation Cdh23^ahl, which on this background causes progressive hearing loss with onset after 10 months of age.

Description
View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for Kcnq2^Nmf134 entry.

Control Information

	Control
	Unaffected littermates of affected mice
Considerations for Choosing Controls

Related Strains

Strains carrying   Cdh23^ahl allele

001137	129P1/ReJ
000690	129P3/J
000691	129X1/SvJ
000646	A/J
000647	A/WySnJ
003070	ALR/LtJ
003072	ALS/LtJ
004502	B6;AKR-Lxl2/GrsrJ
001026	BALB/cByJ
000653	BUB/BnJ
005494	C3.129S1(B6)-Grm1rcw/J
000664	C57BL/6J
004764	C57BL/6J-Cdh23v-8J/J
003129	C57BL/6J-Epha4rb-2J/GrsrJ
004820	C57BL/6J-Kcne12J/J
004811	C57BL/6J-nmf110/J
004812	C57BL/6J-nmf111/J
004747	C57BL/6J-nmf118/J
004656	C57BL/6J-nmf88/J
004391	C57BL/6J-Chr 13A/J/NaJ
004385	C57BL/6J-Chr 7A/J/NaJ
000662	C57BLKS/J
000667	C57BR/cdJ
000668	C57L/J
000669	C58/J
000657	CE/J
000670	DBA/1J
001140	DBA/1LacJ
000671	DBA/2J
007048	DBA/2J-Gpnmb+/SjJ
002106	KK/HlJ
000675	LG/J
000676	LP/J
000677	MA/MyJ
001976	NOD/ShiLtJ
002050	NOR/LtJ
000679	P/J
002747	SENCARB/PtJ
002335	SKH2/J
003392	STOCK Crb1rd8/J

View Strains carrying   Cdh23^ahl     (40 strains)

Strains carrying other alleles of Cdh23

002756	B6.CAST-Cdh23Ahl+/Kjn
002432	B6J x B6.C-H2-Kbm1/ByJ-Cdh23v-J/J
002552	C57BL/6J-Cdh23v-2J/J
004764	C57BL/6J-Cdh23v-8J/J
004819	C57BL/6J-Cdh23v-9J/J
005016	CByJ;B6-Cdh23v-10J/J
000275	V/LeJ

View Strains carrying other alleles of Cdh23     (7 strains)

Strains carrying other alleles of Kcnq2

005830

B6.129P2-Kcnq2tm1Dgen/J

View Strains carrying other alleles of Kcnq2     (1 strain)

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms

      assigned by genotype

Kcnq2^Nmf134/Kcnq2⁺

        C57BL/6J

• behavior/neurological phenotype
• abnormal seizure response to electrical stimulation (MGI Ref ID J:82238)
  • mutants appear to have a low threshold to electroconvulsive clonic seizures. i.e. when stimulated transcorneally for minimal seizures, the majority of mice respond not with minimal, but with intense seizure
  • rearing, forelimb clonus and jaw clonus, and/or tonic hind limb extension defined the intensity of the seizure as high
• nervous system phenotype
• abnormal seizure response to electrical stimulation (MGI Ref ID J:82238)
  • mutants appear to have a low threshold to electroconvulsive clonic seizures. i.e. when stimulated transcorneally for minimal seizures, the majority of mice respond not with minimal, but with intense seizure
  • rearing, forelimb clonus and jaw clonus, and/or tonic hind limb extension defined the intensity of the seizure as high

Kcnq2^Nmf134/Kcnq2⁺

        C57BL/6J-Kcnq2^Nmf134

• behavior/neurological phenotype
• abnormal seizure response to electrical stimulation (MGI Ref ID J:136510)
  • mice exhibit electrically induced seizures
  • however, mice do not exhibit spontaneous seizures
• increased susceptibility to pharmacologically induced seizures (MGI Ref ID J:136510)
  • mice exhibit a reduced threshold to seizures induced by petylenetetrazol compared to wild-type mice
  • however, mice do not exhibit spontaneous seizures
• nervous system phenotype
• *normal* nervous system phenotype (MGI Ref ID J:136510)
  • mice exhibit normal nervous system morphology
• abnormal seizure response to electrical stimulation (MGI Ref ID J:136510)
  • mice exhibit electrically induced seizures
  • however, mice do not exhibit spontaneous seizures
• increased susceptibility to pharmacologically induced seizures (MGI Ref ID J:136510)
  • mice exhibit a reduced threshold to seizures induced by petylenetetrazol compared to wild-type mice
  • however, mice do not exhibit spontaneous seizures

Kcnq2^Nmf134/Kcnq2^Nmf134

        C57BL/6J

• behavior/neurological phenotype
• abnormal seizure response to electrical stimulation (MGI Ref ID J:82238)
  • mice have a low threshold to electroconvulsive clonic seizures. i.e. when stimulated transcorneally for minimal seizures, mice respond not with minimal, but with intense seizure
• nervous system phenotype
• abnormal seizure response to electrical stimulation (MGI Ref ID J:82238)
  • mice have a low threshold to electroconvulsive clonic seizures. i.e. when stimulated transcorneally for minimal seizures, mice respond not with minimal, but with intense seizure

View Research Applications

Research Applications

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

Cdh23^ahl related

Neurobiology Research
Vestibular and Hearing Defects
      Age related hearing loss

Sensorineural Research
Vestibular and Hearing Defects
      Age related hearing loss

Kcnq2^Nmf134 related

Neurobiology Research
Epilepsy
      electroconvulsive seizures
Neuroscience Mutagenesis Facility Strain

Genes & Alleles

Gene & Allele Information

Allele Symbol		Kcnq2Nmf134
Allele Name		neuroscience mutagenesis facility, 134
Allele Type		Chemically induced (ENU)
Common Name(s)		Kcnq2V182M;
Strain of Origin		C57BL/6J
Gene Symbol and Name		Kcnq2, potassium voltage-gated channel, subfamily Q, member 2
Chromosome		2
Gene Common Name(s)		BFNC; EBN; EBN1; ENB1; HNSPC; KCNA11; KQT2; KV7.2; KVEBN1; Nmf134; neuroscience mutagenesis facility, 134;
Molecular Note		A G-to-T transversion mutation in codon 182 results in the substitution of methionine for valine at this highly conserved position in the encoded protein. [MGI Ref ID J:136510]
Allele Symbol		Cdh23ahl
Allele Name		age related hearing loss 1
Allele Type		QTL
Common Name(s)		Cdh23753A; mdfw;

Genotyping

Genotyping Information

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

Helpful Links

Genotyping resources and troubleshooting

References

References

Additional References

Kcnq2^Nmf134 related

JAX Neuroscience Mutagenesis Facility URL: http://www.jax.org/nmf. 2003. Heritable mouse mutants from JAX NMF ENU Mutagenesis Program MGI Direct Data Submission :.  [MGI Ref ID J:82238]

Kearney JA; Yang Y; Beyer B; Bergren SK; Claes L; Dejonghe P; Frankel WN. 2006. Severe epilepsy resulting from genetic interaction between Scn2a and Kcnq2. Hum Mol Genet 15(6):1043-8. [PubMed: 16464983]  [MGI Ref ID J:136510]

Health & husbandry

Health & Colony Maintenance Information

Currently there no information available for this strain. This may be due to the supply level of this strain.

Purchasing information

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

Pricing

Supply Details

Standard Supply	Cryopreserved. Ready for recovery. Please refer to pricing and supply notes 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. This strain is included in the Neuroscience Mutagenesis Facility (NMF).
Important Note
The C57BL/6J background strain is homozygous for the age related hearing loss mutation Cdh23ahl, which on this background causes progressive hearing loss with onset after 10 months of age.

Control Information

	Control
	Unaffected littermates of affected mice
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.

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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.

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