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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
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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 B6C3Fe a/a-Qk/J (Changed: 15-DEC-04 ) B6C3Fe a/a-qk/+ (Changed: 15-DEC-04 ) B6C3Fe-a/a-qk/+ (Changed: 15-DEC-04 ) Type Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation N16p Appearance
black, tremors
Related Genotype: a/a Qkqk/Qkqk
black, unaffected
Related Genotype: a/a Qkqk/+ or a/a ?/+Description
Mice homozygous for the quaking spontaneous mutation (Qk) 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.Development
The quaking (Qkqk) 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 1976 a Qkqk homozygote was outcrossed to a B6C3HF1 male, removing the T2J mutation, and the Qkqk mutation was maintained via cross-intercross using Qkqk homozygous females and B6C3F1 males for the cross since homozygous males are sterile. At N8F4 a change was made to use B6C3Fe-a/a F1 for the outcross. In 1987 homozygous females at N15F1 were bred with B6C3F3-a/a F1 males to generate embryos for cryopreservation.
Control Information
| Control | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying Qkqk allele
000567 B6.Cg-T2J +/+ Qkqk/J View Strains carrying Qkqk (1 strain)
005089 B6.Cg-Qkqk-2J/GrsrJ
Phenotype
Phenotype Information
assigned by genotype
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
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
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
This mouse can be used to support research in many areas including:
Qkqk relatedNeurobiology Research
Parkinson's Disease
Park2 (parkin) mutants
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
Genes & Alleles
Gene & Allele Information
| Allele Symbol | Qkqk | ||
|---|---|---|---|
| Allele Name | quaking | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | qk; qkv; | ||
| Strain of Origin | DBA/2J | ||
| Gene Symbol and Name | Qk, quaking | ||
| Chromosome | 17 | ||
| Gene Common Name(s) | 1110003F05Rik; DKFZp586I0923; Hqk; MGC188205; QK1; QK3; QkI; RIKEN cDNA 1110003F05 gene; 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 | a | ||
| Allele Name | nonagouti | ||
| Allele Type | Spontaneous | ||
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
Selected Reference(s)
SIDMAN RL; DICKIE MM; APPEL SH. 1964. MUTANT MICE (QUAKING AND JIMPY) WITH DEFICIENT MYELINATION IN THE CENTRAL NERVOUS SYSTEM. Science 144:309-11. [PubMed: 14169723] [MGI Ref ID J:13141]
Additional References
Mitrovic N; Caboche J; Carre JB; Besson MJ; Maurin Y. 1991. The quaking mouse: an epileptic mutant with alterations affecting the modulatory mechanisms of the NMDA receptor complex. Brain Res 566(1-2):248-54. [PubMed: 1839963] [MGI Ref ID J:1930]
Yanagimachi R; Wakayama T; Kishikawa H; Fimia GM; Monaco L; Sassone-Corsi P. 2004. Production of fertile offspring from genetically infertile male mice. Proc Natl Acad Sci U S A 101(6):1691-5. [PubMed: 14757819] [MGI Ref ID J:88151]
Qkqk relatedAdham IM; Khulan J; Held T; Schmidt B; Meyer BI; Meinhardt A; Engel W. 2008. Fas-associated factor (FAF1) is required for the early cleavage-stages of mouse embryo. Mol Hum Reprod 14(4):207-13. [PubMed: 18303090] [MGI Ref ID J:135886]
Aguayo AJ; Mizuno K; Bray GM. 1977. Schwann cell transplantation: evidence for a primary sheath cell disorder causing hypomyelination in quaking mice J Neuropathol Exp Neurol 36:595. [MGI Ref ID J:14892]
Barbarese E. 1991. Spatial distribution of myelin basic protein mRNA and polypeptide in quaking oligodendrocytes in culture. J Neurosci Res 29(3):271-81. [PubMed: 1717701] [MGI Ref ID J:1931]
Bartoszewicz ZP; Noronha AB; Fujita N; Sato S; Bo L; Trapp BD; Quarles RH. 1995. Abnormal expression and glycosylation of the large and small isoforms of myelin-associated glycoprotein in dysmyelinating quaking mutants. J Neurosci Res 41(1):27-38. [PubMed: 7545761] [MGI Ref ID J:26581]
Baumann NA; Bourre JM; Jacque C; Pollett S. 1972. Genetic disorders of myelination. In: Lipids, Malnutrition and the Developing Brain. ASP (Elsevier Excerpta Medica, North-Holland, Amsterdam. [MGI Ref ID J:26986]
Baumann NA; Harpin ML; Bourre JM. 1970. Long chain fatty acid formation: key step in myelination studied in mutant mice. Nature 227(261):960-1. [PubMed: 5449004] [MGI Ref ID J:5171]
Bennett WI; Gall AM; Southard JL; Sidman RL. 1971. Abnormal spermiogenesis in quaking, a myelin-deficient mutant mouse. Biol Reprod 5(1):30-58. [PubMed: 5166852] [MGI Ref ID J:5241]
Berger B. 1971. [Some ultrastructural aspects of white matter in Quaking mice] Brain Res 25(1):35-53. [PubMed: 5541257] [MGI Ref ID J:5189]
Billings-Gagliardi S; Adcock LH; Lamperti ED; Schwing-Stanhope G; Wolf MK. 1983. Myelination of jp,jpmsd, and qk axons by normal glia in vitro: ultrastructural and autoradiographic evidence. Brain Res 268(2):255-66. [PubMed: 6871684] [MGI Ref ID J:7126]
Bo L; Quarles RH; Fujita N; Bartoszewicz Z; Sato S; Trapp BD. 1995. Endocytic depletion of L-MAG from CNS myelin in quaking mice. J Cell Biol 131(6 Pt 2):1811-20. [PubMed: 8557747] [MGI Ref ID J:30401]
Bourre JM; Clement M; Gerard D; Chaudiere J. 1989. Alterations of cholesterol synthesis precursors (7-dehydrocholesterol, 7-dehydrodesmosterol, desmosterol) in dysmyelinating neurological mutant mouse (quaking, shiverer and trembler) in the PNS and the CNS. Biochim Biophys Acta 1004(3):387-90. [PubMed: 2547434] [MGI Ref ID J:9907]
Braun PE; Horvath E; Edwards AM. 1990. Two isoforms of myelin-associated glycoprotein accumulate in quaking mice: only the large polypeptide is phosphorylated. Dev Neurosci 12(4-5):286-92. [PubMed: 1705210] [MGI Ref ID J:116790]
Campagnoni AT; Campagnoni CW; Bourre JM; Jacque C; Baumann N. 1984. Cell-free synthesis of myelin basic proteins in normal and dysmyelinating mutant mice. J Neurochem 42(3):733-9. [PubMed: 6198470] [MGI Ref ID J:7310]
Campagnoni CW; Garbay B; Micevych P; Pribyl T; Kampf K; Handley VW; Campagnoni AT. 1992. DM20 mRNA splice product of the myelin proteolipid protein gene is expressed in the murine heart. J Neurosci Res 33(1):148-55. [PubMed: 1280689] [MGI Ref ID J:3020]
Chen H; Sun P; Parmantier E; Cabon F; Dupouey P; Zalc B; Jacque C. 1992. Developmental expression of glial fibrillary acidic protein and actin-encoding messages in quaking and control mice. Dev Neurosci 14(5-6):351-6. [PubMed: 1306160] [MGI Ref ID J:13328]
Chubb C. 1992. Oligotriche and quaking gene mutations. Phenotypic effects on mouse spermatogenesis and testicular steroidogenesis. J Androl 13(4):312-7. [PubMed: 1399832] [MGI Ref ID J:12215]
Cox RD; Hugill A; Shedlovsky A; Noveroske JK; Best S; Justice MJ ; Lehrach H ; Dove WF. 1999. Contrasting effects of ENU induced embryonic lethal mutations of the quaking gene. Genomics 57(3):333-41. [PubMed: 10328999] [MGI Ref ID J:55007]
Dapper JD; Justice MJ. 2005. Defining the breakpoints of the quaking(viable) mouse mutation reveals a duplication from a Parkin intron. Mov Disord 20(10):1369-74. [PubMed: 16001410] [MGI Ref ID J:101474]
DeWille JW; Farmer SJ. 1992. Quaking phenotype influences brain lipid-related mRNA levels. Neurosci Lett 141(2):195-8. [PubMed: 1279471] [MGI Ref ID J:3783]
Dev A; Nayernia K; Meins M; Adham I; Lacone F; Engel W. 2007. Mice deficient for RNA-binding protein brunol1 show reduction of spermatogenesis but are fertile. Mol Reprod Dev 74(11):1456-64. [PubMed: 17393433] [MGI Ref ID J:128362]
Ebersole TA; Chen Q; Justice MJ; Artzt K. 1996. The quaking gene product necessary in embryogenesis and myelination combines features of RNA binding and signal transduction proteins [see comments] Nat Genet 12(3):260-5. [PubMed: 8589716] [MGI Ref ID J:31757]
Fagg GE. 1979. The quaking mouse: regional variations in the content and protein composition of myelin isolated from the central nervous system. Neuroscience 4(7):973-8. [PubMed: 552615] [MGI Ref ID J:6411]
Frail DE; Braun PE. 1985. Abnormal expression of the myelin-associated glycoprotein in the central nervous system of dysmyelinating mutant mice. J Neurochem 45(4):1071-5. [PubMed: 2411865] [MGI Ref ID J:7990]
Friedrich VL Jr. 1975. Hyperplasia of oligodendrocytes in quaking mice. Anat Embryol (Berl) 147(3):259-71. [PubMed: 174456] [MGI Ref ID J:5615]
Fujita N; Sato S; Kurihara T; Inuzuka T; Takahashi Y; Miyatake T. 1988. Developmentally regulated alternative splicing of brain myelin-associated glycoprotein mRNA is lacking in the quaking mouse. FEBS Lett 232(2):323-7. [PubMed: 2454205] [MGI Ref ID J:38791]
Greenfield S; Williams NI; White M; Brostoff SW; Hogan EL. 1979. Proteolipid protein: synthesis and assembly into quaking mouse myelin. J Neurochem 32(6):1647-51. [PubMed: 448358] [MGI Ref ID J:6151]
Hardy RJ. 1998. Molecular defects in the dysmyelinating mutant quaking. J Neurosci Res 51(4):417-22. [PubMed: 9514195] [MGI Ref ID J:46482]
Hardy RJ; Loushin CL; Friedrich VL Jr; Chen Q; Ebersole TA; Lazzarini RA; Artzt K. 1996. Neural cell type-specific expression of QKI proteins is altered in quakingviable mutant mice. J Neurosci 16(24):7941-9. [PubMed: 8987822] [MGI Ref ID J:37139]
Jacque C; Delassalle A; Raoul M; Baumann N. 1983. Myelin basic protein deposition in the optic and sciatic nerves of dysmyelinating mutants quaking, jimpy, Trembler, mld, and shiverer during development. J Neurochem 41(5):1335-40. [PubMed: 6194264] [MGI Ref ID J:12030]
Jones SM; Johnson KR; Yu H; Erway LC; Alagramam KN; Pollak N; Jones TA. 2005. A quantitative survey of gravity receptor function in mutant mouse strains. J Assoc Res Otolaryngol 6(4):297-310. [PubMed: 16235133] [MGI Ref ID J:116914]
King TR; Dove WF. 1991. Pleiotropic action of the murine quaking locus: structure of the qkv allele. Mamm Genome 1(1):47-52. [PubMed: 1665374] [MGI Ref ID J:11620]
Kirschner DA; Sidman RL. 1976. X-ray diffraction study of myelin structure in immature and mutant mice. Biochim Biophys Acta 448(1):73-87. [PubMed: 971429] [MGI Ref ID J:5688]
Konat G; Trojanowska M; Gantt G; Hogan EL. 1988. Expression of myelin protein genes in quaking mouse brain. J Neurosci Res 20(1):19-22. [PubMed: 3418751] [MGI Ref ID J:31025]
Kraszucka K; Burfeind P; Nayernia K; Kohler M; Schmid M; Yaylaoglu M; Engel W. 1999. Developmental stage- and germ cell-regulated expression of a calcium-binding protein mRNA in mouse Sertoli cells. Mol Reprod Dev 54(3):232-43. [PubMed: 10497345] [MGI Ref ID J:57920]
Kuchler S; Zanetta JP; Zaepfel M; Badache A; Sarlieve LL; Gumpel M; Baumann N; Vincendon G. 1990. Endogenous cerebellar soluble lectin and its ligands in central nervous system myelin of quaking and jimpy mutant mice. Dev Neurosci 12(6):382-97. [PubMed: 2076671] [MGI Ref ID J:116788]
Kurihara T; Takahashi Y; Fujita N; Sato S; Miyatake T. 1989. Developmental expression of 2',3'-cyclic-nucleotide 3'-phosphodiesterase mRNA in brains of normal and quaking mice. Brain Res Mol Brain Res 5(3):247-50. [PubMed: 2542718] [MGI Ref ID J:1352]
Larocque D; Pilotte J; Chen T; Cloutier F; Massie B; Pedraza L; Couture R; Lasko P; Almazan G; Richard S. 2002. Nuclear retention of MBP mRNAs in the quaking viable mice. Neuron 36(5):815-29. [PubMed: 12467586] [MGI Ref ID J:80746]
Le Saux F; Besson MJ; Maurin Y. 2002. Abnormal postnatal ontogeny of the locus coeruleus in the epileptic mutant mouse quaking. Brain Res Dev Brain Res 136(2):197-205. [PubMed: 12101037] [MGI Ref ID J:109168]
LeVine SM. 1991. Oligodendrocytes and myelin sheaths in normal, quaking and shiverer brains are enriched in iron. J Neurosci Res 29(3):413-9. [PubMed: 1920537] [MGI Ref ID J:1932]
LeVine SM; Brown DC. 1997. IL-6 and TNFalpha expression in brains of twitcher, quaking and normal mice. J Neuroimmunol 73(1-2):47-56. [PubMed: 9058758] [MGI Ref ID J:40116]
Li WX; Kuchler S; Zaepfel M; Badache A; Thomas D; Vincendon G; Baumann N; Zanetta JP. 1993. Cerebellar soluble lectin and its glycoprotein ligands in the developing brain of control and dysmyelinating mutant mice. Neurochem Int 22(2):125-33. [PubMed: 8439766] [MGI Ref ID J:4577]
Li Z; Zhang Y; Li D; Feng Y. 2000. Destabilization and mislocalization of myelin basic protein mRNAs in quaking dysmyelination lacking the QKI RNA-binding proteins. J Neurosci 20(13):4944-53. [PubMed: 10864952] [MGI Ref ID J:63120]
Lindsey JS; Wilkinson MF. 1996. Pem: a testosterone- and LH-regulated homeobox gene expressed in mouse Sertoli cells and epididymis. Dev Biol 179(2):471-84. [PubMed: 8903361] [MGI Ref ID J:36481]
Lockhart PJ; O'Farrell CA; Farrer MJ. 2004. It's a double knock-out! The quaking mouse is a spontaneous deletion of parkin and parkin co-regulated gene (PACRG). Mov Disord 19(1):101-4. [PubMed: 14743368] [MGI Ref ID J:87498]
Lorenzetti D; Antalffy B; Vogel H; Noveroske J; Armstrong D; Justice M. 2004. The neurological mutant quaking(viable) is Parkin deficient. Mamm Genome 15(3):210-7. [PubMed: 15014970] [MGI Ref ID J:88351]
Lorenzetti D; Bishop CE; Justice MJ. 2004. Deletion of the Parkin coregulated gene causes male sterility in the quaking(viable) mouse mutant. Proc Natl Acad Sci U S A 101(22):8402-7. [PubMed: 15148410] [MGI Ref ID J:90667]
Lu Z; Ku L; Chen Y; Feng Y. 2005. Developmental abnormalities of myelin basic protein expression in fyn knock-out brain reveal a role of Fyn in posttranscriptional regulation. J Biol Chem 280(1):389-95. [PubMed: 15528192] [MGI Ref ID J:104981]
Lu Z; Zhang Y; Ku L; Wang H; Ahmadian A; Feng Y. 2003. The quakingviable mutation affects qkI mRNA expression specifically in myelin-producing cells of the nervous system. Nucleic Acids Res 31(15):4616-24. [PubMed: 12888522] [MGI Ref ID J:84952]
Mateu L; Luzzati V; Vonasek E; Borgo M; Lachapelle F. 1996. Order-disorder phenomena in myelinated nerve sheaths. VI. The effects of quaking, jimpy and shiverer mutations: an X-ray scattering study of mouse sciatic and optic nerves. J Mol Biol 256(2):319-29. [PubMed: 8594199] [MGI Ref ID J:31918]
Mikoshiba K; Nagaike K; Aoki E; Tsukada Y. 1979. Biochemical and immunohistochemical studies on dysmyelination of quaking mutant mice in vivo and in vitro. Brain Res 177(2):287-99. [PubMed: 227533] [MGI Ref ID J:6216]
Mitrovic N; Caboche J; Carre JB; Besson MJ; Maurin Y. 1991. The quaking mouse: an epileptic mutant with alterations affecting the modulatory mechanisms of the NMDA receptor complex. Brain Res 566(1-2):248-54. [PubMed: 1839963] [MGI Ref ID J:1930]
Mitrovic N; Le Saux F; Gioanni H; Gioanni Y; Besson MJ; Maurin Y. 1992. Distribution of [3H]clonidine binding sites in the brain of the convulsive mutant quaking mouse: a radioautographic analysis. Brain Res 578(1-2):26-32. [PubMed: 1511279] [MGI Ref ID J:1169]
Moutier R. 1973. t Mouse News Lett 49:42. [MGI Ref ID J:15078]
Murad S; Kishimoto Y. 1975. Alpha hydroxylation of lignoceric acid to cerebronic acid during brain development. Diminished hydroxylase activity in myelin-deficient mouse mutants. J Biol Chem 250(15):5841-6. [PubMed: 1150661] [MGI Ref ID J:106960]
Nikulina EM; Skrinskaya JA; Avgustinovich DF; Popova NK. 1995. Dopaminergic brain system in the quaking mutant mouse. Pharmacol Biochem Behav 50(3):333-7. [PubMed: 7617670] [MGI Ref ID J:24440]
Nixon RA. 1982. Increased axonal proteolysis in myelin-deficient mutant mice. Science 215(4535):999-1001. [PubMed: 7156980] [MGI Ref ID J:6971]
Noveroske JK; Hardy R; Dapper JD; Vogel H; Justice MJ. 2005. A new ENU-induced allele of mouse quaking causes severe CNS dysmyelination. Mamm Genome 16(9):672-82. [PubMed: 16245024] [MGI Ref ID J:102038]
Nussbaum JL; Mandel P. 1973. Brain proteolipids in neurological mutant mice. Brain Res 61:295-310. [PubMed: 4129706] [MGI Ref ID J:5408]
Propst F; Rosenberg MP; Oskarsson MK; Russell LB; Nguyen-Huu MC; Nadeau J; Jenkins NA; Copeland NG; Vande Woude GF. 1988. Genetic analysis and developmental regulation of testis-specific RNA expression of Mos, Abl, actin and Hox-1.4. Oncogene 2(3):227-33. [PubMed: 2895445] [MGI Ref ID J:9103]
Rawal N; Lee YJ; Paik WK; Kim S. 1992. Studies on NG-methylarginine derivatives in myelin basic protein from developing and mutant mouse brain. Biochem J 287(Pt 3):929-35. [PubMed: 1280107] [MGI Ref ID J:3367]
Rosenfeld J; Zimmerman AW; Friedrich VL Jr. 1983. Altered brain copper and zinc content in quaking mice. Exp Neurol 82(1):55-63. [PubMed: 6628615] [MGI Ref ID J:7214]
Samorajski T; Friede RL; Reimer PR. 1970. Hypomyelination in the quaking mouse. A model for the analysis of disturbed myelin formation. J Neuropathol Exp Neurol 29(4):507-23. [PubMed: 5471919] [MGI Ref ID J:5177]
Shedlovsky A; King TR; Dove WF. 1988. Saturation germ line mutagenesis of the murine t region including a lethal allele at the quaking locus. Proc Natl Acad Sci U S A 85(1):180-4. [PubMed: 3422415] [MGI Ref ID J:9032]
Sidman M; Ray BA; Sidman RL; Klinger JM. 1966. Hearing and vision in neurological mutant mice: a method for their evaluation. Exp Neurol 16(4):377-402. [PubMed: 5957201] [MGI Ref ID J:94552]
Sorg BA; Smith MM; Campagnoni AT. 1987. Developmental expression of the myelin proteolipid protein and basic protein mRNAs in normal and dysmyelinating mutant mice. J Neurochem 49(4):1146-54. [PubMed: 2442307] [MGI Ref ID J:28225]
Suzuki K; Zagoren JC. 1977. Quaking mouse: an ultrastructural study of the peripheral nerves. J Neurocytol 6(1):71-84. [PubMed: 190360] [MGI Ref ID J:5778]
Tucker TA; Kundert JA; Bondareva AA; Schmidt EE. 2005. Reproductive and neurological Quakingviable phenotypes in a severe combined immune deficient mouse background. Immunogenetics 57(3-4):226-31. [PubMed: 15900494] [MGI Ref ID J:98496]
Watanabe I; Bingle GJ. 1972. Dysmyelination in quaking mouse. Electron microscopic study. J Neuropathol Exp Neurol 31(2):352-69. [PubMed: 5026019] [MGI Ref ID J:5271]
Wisniewski H; Morell P. 1971. Quaking mouse: ultrastructural evidence for arrest of myelinogenesis. Brain Res 29(1):63-73. [PubMed: 5564263] [MGI Ref ID J:5218]
Wolf MK; Nunnari JN; Billings-Gagliardi S. 1999. Quaking*shiverer double-mutant mice survive for at least 100 days with no CNS myelin. Dev Neurosci 21(6):483-90. [PubMed: 10640866] [MGI Ref ID J:116376]
Yanagimachi R; Wakayama T; Kishikawa H; Fimia GM; Monaco L; Sassone-Corsi P. 2004. Production of fertile offspring from genetically infertile male mice. Proc Natl Acad Sci U S A 101(6):1691-5. [PubMed: 14757819] [MGI Ref ID J:88151]
Zhang Y; Feng Y. 2001. Distinct molecular mechanisms lead to diminished myelin basic protein and 2',3'-cyclic nucleotide 3'-phosphodiesterase in qk(v) dysmyelination. J Neurochem 77(1):165-72. [PubMed: 11279272] [MGI Ref ID J:68451]
Zhao L; Tian D; Xia M; Macklin WB; Feng Y. 2006. Rescuing qkV dysmyelination by a single isoform of the selective RNA-binding protein QKI. J Neurosci 26(44):11278-86. [PubMed: 17079655] [MGI Ref ID J:114709]
van Abeelen JH. 1966. Behavioural profiles of neurological mutant mice. Genetica 37(2):149-58. [PubMed: 5955164] [MGI Ref ID J:133042]
Health & husbandry
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.
Health & Colony Maintenance Information
Colony Maintenance
Breeding & Husbandry Comments: homozygous males are sterile.
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Pricing, Supply Level & Notes, Controls, General Terms & Conditions
Pricing
| Pricing for USA, Canada and Mexico shipping destinations |
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Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 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.
Additional Supply Details
| Pricing for International shipping destinations |
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Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 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.
Additional Supply Details
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Supply Details
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
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| Supply Notes |
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Control Information
| Control | ||
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| Untyped from the colony | ||
| 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 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.Ordering and Purchasing Information
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JAX® Mice, Products & Services Conditions of Use
"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.No Warranty
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The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.
Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.