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Strain Name:

B6.Cg-T2J +/+ Qkqk/J

Stock Number:

000567

Availability:

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General Terms and Conditions

Former Name      B6.Cg-T2J +/+ Qk/J    (Changed: 15-DEC-04 )
      B6.Cg-T2J +/+ qk    (Changed: 15-DEC-04 )
Genes & Alleles   Qk;   Qkqk;   T;   T2J;

Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain Qkqk DBA/2J; T2J , C57BL/6J
GenerationN18 F20p

Strain Description
Mice homozygous for the quaking spontaneous mutation (Qkqk) have marked rapid tremor which disappears when they are at rest but increases during locomotion. The tremor in homozygous mutant mice begins at about 10 days and is fully developed by 3 weeks. Mature mice may have seizures in which a motionless posture is maintained for many seconds. Females are viable and fertile, males are sterile due to defective spermatic differentiation. The entire CNS of quaking mutant mice is severely deficient in myelin and there is a less severe myelin deficiency in the PNS. In this strain the brachyury 2 Jackson mutation (T2J) is maintained in repulsion with the quaking mutation.

Strain Development
The quaking (qk) mutation arose spontaneously in 1961 in the DBA/2J strain. It was crossed twice to C3H then transferred to the C57BL/6JEi background via backcross-intercross mating until N10 then sibling bred. At N11F10 it was bred to C57BL/6J-T2J, and a repulsion stock was generated. In 1994 T2J +/+ Qkqk females were bred with T2J +/+ Qkqk males, all at or beyond N18F19, to generate embryos for cryopreservation.

Mammalian Phenotype Terms assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Qkqk/Qkqk

        involves: DBA/2J
  • behavior/neurological phenotype
  • abnormal response to novel object (MGI Ref ID J:133042)
    • decrease in the frequency of exploratory sniffing (at wire mesh or a novel object) and leans against the novel object in males
  • abnormal voluntary movement (MGI Ref ID J:133042)
    • decrease in the frequency of exploratory sniffing (at wire mesh or a novel object) in males
    • abnormal locomotor activity (MGI Ref ID J:133042)
      • ecrease in the frequency of wire mesh climbing in males
      • abnormal gait (MGI Ref ID J:133042)
        • walk slowly with a trembling gait
      • hypoactivity (MGI Ref ID J:133042)
    • abnormal stationary movement (MGI Ref ID J:133042)
      • decrease in the frequency of leaning against the wall or a novel object and single forepaw lifts in males
  • increased grooming behavior (MGI Ref ID J:133042)
    • increase in the frequency of hair fluffing in males

Qkqk/Qkqk

        B6C3Fe a/a-Qkqk/J
  • hearing/vestibular/ear phenotype
  • reduced linear vestibular evoked potential (MGI Ref ID J:116914)
    • prolonged latency for all peaks and larger amplitudes for P1/N1
  • nervous system phenotype
  • Purkinje cell degeneration (MGI Ref ID J:102038)
    • aging mutants exhibit Purkinje cell axonal swellings, indicating neurodegeneration
  • abnormal myelination (MGI Ref ID J:102038)
    • decrease in myelination, however more axons are surrounded by thin myelin sheaths than seen in Qke5 homozygotes
  • seizures (MGI Ref ID J:102038)
    • onset of seizures begins at 6-8 weeks of age; seizures occur less frequently than in homozygous Qke5 mice
  • behavior/neurological phenotype
  • seizures (MGI Ref ID J:102038)
    • onset of seizures begins at 6-8 weeks of age; seizures occur less frequently than in homozygous Qke5 mice

Qkqk/Qkqk

        involves: C3H/Di * DBA/2J
  • nervous system phenotype
  • abnormal myelination (MGI Ref ID J:13141)
    • region from olfactory bulb to sacral spinal cord is deficient in myelin at all ages studied (12 days to 4 months)
    • loss of myelination begins at the junction of peripheral and central nervous systems
    • cranial and spinal nerves (except optic nerve) are myelinated
    • some fragments of myelin are seen in almost all fiber tracts
    • cells in white matter and grey matter tracts appear normal
  • tonic seizures (MGI Ref ID J:13141)
    • following swimming, some mice become motionless and then display a tonic extension of hindlimbs for several minutes
    • some mice exhibit adduction of limbs under a flexed trunk and then become stiff and motionless for several seconds
  • behavior/neurological phenotype
  • tonic seizures (MGI Ref ID J:13141)
    • following swimming, some mice become motionless and then display a tonic extension of hindlimbs for several minutes
    • some mice exhibit adduction of limbs under a flexed trunk and then become stiff and motionless for several seconds
  • tremors (MGI Ref ID J:13141)
    • tremors are most evident in the caudal part of the trunk and proximal portions of hind extremities
    • visually, the rate of tremors are 2 to 3 per second
    • tremors are first observed at 10-12 days of age and reach full expression by 3 weeks
    • in some animals, tremors diminish at 3 months
    • physical contact with the mouse reduces or stops tremors
  • muscle phenotype
  • muscle weakness (MGI Ref ID J:13141)
    • some animals exhibit hindlimb weakness at 3 months of age
  • reproductive system phenotype
  • reduced male fertility (MGI Ref ID J:13141)
    • male homozygotes rarely sire offspring

Gene & Allele Details

Allele Symbol Qkqk
Allele Name quaking
Common Name(s) qk; qkv;
Strain of OriginDBA/2J
Gene Symbol and Name Qk, quaking
Chromosome 17
Gene Common Name(s) DKFZp586I0923; Hqk; QK1; QK3; QkI; l(17)-1Wis; l17Wis1; lethal, Chr 17, U Wisconsin 1;
General Note The quaking mutation arose spontaneously in the DBA/2J strain. Homozygotes have marked rapid tremor which disappears when they are at rest but increases during locomotion. It begins at about 10 days and is fully developed by 3 weeks. Mature mice may haveseizures in which a motionless posture is maintained for many seconds. Females are viable and fertile, males sterile.Homozygotes are severely deficient in myelin, the material which ensheathes and insulates the axons of the central (CNS) and peripheral(PNS) nervous systems (see Mbp). The entire CNS is very deficient in myelin at all ages (J:13141), and there is a less severe myelin deficiency in the PNS nervous system (J:5177). Myelin sheaths are present in the CNS, but they are thinner than normal, some consisting of only one to four myelin lamellae. The sheaths are usually loosely wound, with patches of oligodendroglial cell cytoplasm between the lamellae, and there are abnormal inclusions and vacuoles in the processes and perikarya of oligodendrocytes. Development of the myelin sheaths appears to be arrested in a stage characteristic of very young animals (J:5189)(J:5271)(J:5218). There is variable hyperplasia of oligodendrocytes, greatest in the tracts with the greatest degree of myelination (J:5615). Axons have normal morphology but there is abnormally high proteolysis in the axons of the optic nerve (J:6971). There is evidence that the myelination defect in the CNS is due to defective oligodendrocytes (J:6216).Handling-induced convulsive seizures in qk/qk mice can be inhibited by administration of N-methyl-D-aspartate (NMDA) antagonists. Modulatory mechanisms for the NMDA receptor complex may differ in qk/qk mice from wild-type (J:1930). a2-adrenoceptor (A2A) antagonists also inhibit these seizures, while A2A agonists potentiate them. qk/qk mice have increased brain binding sites for A2A agonists (J:1169).In the PNS, thinly myelinated and unmyelinated fibers have been described in the sciatic nerve and in the intracranial portion of the trigeminal nerve (J:5189)(J:5271). The sheaths may be structurally abnormal with regions of uncompacted myelin lamellae similar to those of the CNS (J:5778). Orthotopic transplantation of pieces of sciatic nerve between quaking and normal mice has shown that the genetic defect is expressed in Schwann cells (J:14892). Qk causes defective myelinogenesis in both oligodendrocytes and Schwann cells (J:6411).There is an extensive literature on biochemical defects related to the deficiency of myelin in quaking mice (J:26986), a consistent finding of which is a severe deficiency of the myelin lipids, sphingomyelin, cerebrosides, and sulfatides, particularly those containing long-chain fatty acids. The normal increase in these fatty acids which occurs between 15 and20 days does not occur in qk/qk mice, so that adult mutants tend to resemble very young controls (J:5171). Brain proteolipids in adult quaking mice retain the relative proportions found in 10-day controls (J:5408). The myelin-associated glycoproteins of different molecular weight in the brains of quaking mice 15 days of age and older are expressed in abnormal proportions (J:7990). Synthesis of myelin basic protein and proteolipids is normal in quaking brains but their incorporation into myelin is defective (J:6151). mRNAs for myelin basic protein, for example, occur in oligodendrocyte cell bodies, but not in the cell processes that actually form the myelin sheath, in qk/qk brain (J:1931). Quaking mice may have abnormal levels of copper and zinc in the brain, but the evidence on this point is conflicting (J:7214).The sterility of male qk/qk mice is due to defective spermatid differentiation, the details of which have been described (J:5241).
Molecular Note The quaking phenotype has been attributed to a 1.85 Mb deletion on chromosome 17. The proximal breakpoint was located in the promoter region of the Qk gene and affects transcript levels of that gene. The distal breakpoint lies between exons 5 and 6 ofthe parkin gene. Both the parkin gene and another co-regulated gene, Pacrg, are inactivated. Although parkin is not expressed in these mutants, the described phenotype appears due to to the defect in Qk expression. [MGI Ref ID J:101474] [MGI Ref ID J:55007] [MGI Ref ID J:87498] [MGI Ref ID J:88351]
 
Allele Symbol T2J
Allele Name brachyury 2 Jackson
Strain of OriginC57BL/6J
Gene Symbol and Name T, brachyury
Chromosome 17
Gene Common Name(s) Bra; Low; Lr; MGC104817; T1; TFT; Tl2; Tl3; brachyury-like 2; brachyury-like 3; cou; coupe; low ratio; me75;
Molecular Note Pulsed-field gel electrophoresis revealed an altered restriction fragment size consistent with a deletion of 80-110kb. [MGI Ref ID J:21263]

Related Strains

Strains carrying   Qkqk allele
000506   B6C3Fe a/a-Qkqk/J
View Strains carrying   Qkqk     (1 strain)

Strains carrying   T2J allele
000545   C57BL/6J-T2J/J
View Strains carrying   T2J     (1 strain)

Strains carrying other alleles of Qk
005089   B6.Cg-Qkqk-2J/J
View Strains carrying other alleles of Qk     (1 strain)

Strains carrying other alleles of T
003879   B10;TFLe-a/a T tf/+ tf/J
000405   B10ScSn.Cg-T/J
004591   B6 x B6Ei.Cg-TWis/EiJ
000953   B6 x BALB/cBy-T4J/J
001518   B6 x STOCK T tf/th45 tf/J
001015   B6.Cg-T4Or/J
001054   B6.Cg-TOrl/EiJ
000350   B6By.Cg-KitW-v MitfMi-wh T/J
002282   BTBR T+ tf/J
001053   C3Sn.AK-Thp/EiJ
001199   C57BL/6J-T5J/J
001961   C57BL/6JEi x STOCK T T(16;17)43H/+ T(16;17)43H/Ei
001814   STOCK Tc/J
View Strains carrying other alleles of T     (13 strains)

Additional Web Information

Congenic Nomenclature

Research Applications

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

Neurobiology Research
Parkinson's Disease (Park2 (parkin) mutants)

Qkqk related

Apoptosis Research

Cell Biology Research
Cell Cycle Regulation
Protein Processing (degradation)

Neurobiology Research
Epilepsy
Myelination Defects
Neurodegeneration
Parkinson's Disease
Tremor Defects
Vestibular and Hearing Defects

Reproductive Biology Research
Developmental Defects Affecting Gonads (males only)
Fertility Defects (males only)

Sensorineural Research
Vestibular and Hearing Defects

T2J related

Developmental Biology Research
Skeletal Defects

References

Additional References

Price and Supply Information

Strain Name: B6.Cg-T2J +/+ Qkqk/J
Stock Number: 000567

Price Details

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Supply Details

Standard SupplyRepository-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; Email: jaxservices@jax.org.

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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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