Strain Name:

B6CBACa Aw-J/A-Kcnj6wv/J

Stock Number:

000247

Availability:

Cryopreserved - Ready for recovery

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 B6CBACa-Aw-J/A-Kcnj6wv    (Changed: 15-DEC-04 )
Type Mutant Strain; Spontaneous Mutation;
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Specieslaboratory mouse
GenerationN31

Appearance
white-bellied agouti, ataxic
Related Genotype: Aw-J/? Kcnj6wv/Kncj6wv

agouti, ataxic
Related Genotype: A/A Kcnj6wv/Kncj6wv

white-bellied agouti, unaffected
Related Genotype: Aw-J/? +/? or Aw-J/A Kncj6wv/+

agouti, unaffected
Related Genotype: A/A +/? or A/A Kncj6wv/+

Description
Mice homozygous for the weaver spontaneous mutation (Kcnj6wv) are recognizable in the second postnatal week by their small size, instability of gait, weakness, and hypotonia. Many homozygous mutant mice die at weaning age, but some survive to adulthood, and females may breed. The cerebellum in homozygous mutants is very small, simple, and almost devoid of granule cells, which degenerate during the second week. Heterozygotes behave normally, but they have a smaller than normal cerebellum with a deficiency of granule cells, some of which fail to migrate into the internal granule layer and remain scattered in the molecular layer. Evidence from cultures of mutant and normal cerebellum show that granule cells of Kcnj6wv/Kcnj6wv and Kcnj6wv/+ mice have gene-dosage dependent abnormalities in morphology and cell behavior. Studies using homozygous weaver/wildtype chimeras indicate that the migration defect of granule cells is intrinsic to the granule cells themselves. The disarrangement of Purkinje cells, however, is not caused by intrinsic action of weaver in these cells. Cell mixing experiments also suggest Kcnj6wv acts non-autonomously and encodes a membrane-associated ligand that induces external germinal layer neuron differentiation.

Development
Weaver (Kcnj6wv) arose spontaneously in the pedigreed expansion stocks of C57BL/6J at The Jackson Laboratory in 1961. It was maintained by mating tested pairs until F21. Ovarian transplantation to C57BL/6J was used to F21N3 and then because of poor breeding performance a weaver (wv/wv) male was outcrossed to a CBA/Ca female and the tested offspring (wv/+) were mated to the B6CBACa hybrids. Backcrossing to the hybrid B6CBACa was then made each generation by mating a homozygous female weaver to a hybrid and mating the known heterozygotes. The stock was at N46 in 1992. It was cryopreserved in 1986 by mating heterozygous females to heterozygous males at N30-N32 or by mating homozygous females to B6CBACa F1 males.

Control Information

  Control
   Untyped from the colony
 
  Considerations for Choosing Controls

Related Strains

View Strains carrying   Aw-J     (31 strains)

Strains carrying other alleles of a
003301   (C57BL/6J x C3H-Eya1bor)F1/J
000251   AEJ.Cg-ae +/a Gdf5bp-H/J
000202   AEJ/Gn-bd/J
000199   AEJ/GnLeJ
000433   B10.C-H3c H13? A/(28NX)SnJ
000427   B10.CE-H13b Aw/(30NX)SnJ
000423   B10.KR-H13? A/SnJ
000420   B10.LP-H13b Aw/Sn
000477   B10.PA-Pldnpa H3e at/SnJ
000419   B10.UW-H3b we Pax1un at/SnJ
003879   B10;TFLe-a/a T tf/+ tf/J
001538   B6 x B6C3Sn a/A-T(1;9)27H/J
000916   B6 x B6C3Sn a/A-T(5;12)31H/J
000602   B6 x B6C3Sn a/A-T(8;16)17H/J
002083   B6 x B6EiC3 a/A-T(7;16)235Dn/J
000507   B6 x B6EiC3 a/A-Otcspf/J
003759   B6 x B6EiC3Sn a/A-T(10;16)232Dn/J
002071   B6 x B6EiC3Sn a/A-T(11;17)202Dn/J
002113   B6 x B6EiC3Sn a/A-T(11A2;16B3)238Dn/J
002068   B6 x B6EiC3Sn a/A-T(11B1;16B5)233Dn/J
002069   B6 x B6EiC3Sn a/A-T(14E4or5;16B5)225Dn/J
001926   B6 x B6EiC3Sn a/A-T(15;16)198Dn/J
001832   B6 x B6EiC3Sn a/A-T(15E;16B1)60Dn/J
003758   B6 x B6EiC3Sn a/A-T(16C3-4;17A2)65Dn/J
001833   B6 x B6EiC3Sn a/A-T(1C2;16C3)45Dn/J
001903   B6 x B6EiC3Sn a/A-T(6F;18C)57Dn/J
001535   B6 x B6EiC3Sn a/A-T(8A4;12D1)69Dn/J
001831   B6 x B6EiC3Sn a/A-T(8C3;16B5)164Dn/J
000618   B6 x FSB/GnEi a/a Ctslfs/J
000577   B6 x STOCK a Oca2p Hps5ru2 Ednrbs/J
000601   B6 x STOCK a/a T(7;18)50H/J
000592   B6 x STOCK T(2;4)13H a/J
000769   B6.C/(HZ18)By-at-44J/J
000001   B6.C3 A/a Mgrn1md/J
000203   B6.C3-Aiy/a/J
000017   B6.C3-Avy/J
001572   B6.C3-am-J/J
000628   B6.CE-A Amy1b Amy2a5b/J
000021   B6.Cg-Ay/J
000231   B6;C3Fe a/a-Csf1op/J
004200   B6;CBACa Aw-J/A-Npr2cn-2J/GrsrJ
000785   B6;D2-a Es1e/EiJ
000604   B6C3 a/A-T(10;13)199H +/+ Lystbg-J/J or Lystbg-2J/J
002807   B6C3Fe a/a-Meox2fla/J
000224   B6C3Fe a/a-Scyl1mdf/J
001037   B6C3Fe a/a-Agtpbp1pcd/J
000221   B6C3Fe a/a-Alx4lst-J/J
002062   B6C3Fe a/a-Atp7aMo-8J/J
001756   B6C3Fe a/a-Cacng2stg/J
001815   B6C3Fe a/a-Col1a2oim/J
000209   B6C3Fe a/a-Dh/J
000211   B6C3Fe a/a-Dstdt-J/J
000210   B6C3Fe a/a-Edardl-J/J
000207   B6C3Fe a/a-Edaraddcr/J
000182   B6C3Fe a/a-Eef1a2wst/J
001278   B6C3Fe a/a-Glra1spd/J
000241   B6C3Fe a/a-Glrbspa/J
002875   B6C3Fe a/a-Hoxd13spdh/J
000304   B6C3Fe a/a-Krt71Ca Scn8amed-J/J
000226   B6C3Fe a/a-Largemyd/J
000636   B6C3Fe a/a-Lmx1adr-J/J
001280   B6C3Fe a/a-Lse/J
001573   B6C3Fe a/a-MitfMi/J
001035   B6C3Fe a/a-Napahyh/J
000181   B6C3Fe a/a-Otogtwt/J
000278   B6C3Fe a/a-Papss2bm Hps1ep Hps6ru/J
000205   B6C3Fe a/a-Papss2bm/J
002078   B6C3Fe a/a-Pcdh15av-2J/J
000246   B6C3Fe a/a-Pitpnavb/J
001430   B6C3Fe a/a-Ptch1mes/J
000506   B6C3Fe a/a-Qkqk/J
000235   B6C3Fe a/a-Relnrl/J
000237   B6C3Fe a/a-Rorasg/J
000290   B6C3Fe a/a-Sox10Dom/J
000230   B6C3Fe a/a-Tcirg1oc/J
003612   B6C3Fe a/a-Trak1hyrt/J
001512   B6C3Fe a/a-Ttnmdm/J
001607   B6C3Fe a/a-Unc5crcm/J
000005   B6C3Fe a/a-Wc/J
000243   B6C3Fe a/a-Wnt1sw/J
000248   B6C3Fe a/a-Xpl/J
001750   B6C3Fe a/a-XsJ/J
000624   B6C3Fe a/a-anx/J
008044   B6C3Fe a/a-bpck/J
003020   B6C3Fe a/a-dep/J
002018   B6C3Fe a/a-din/J
002339   B6C3Fe a/a-nma/J
000240   B6C3Fe a/a-soc/J
000063   B6C3Fe a/a-sy/J
001055   B6C3Fe a/a-tip/J
000245   B6C3Fe a/a-tn/J
000065   B6C3Fe a/a-we Pax1un at/J
000296   B6C3Fe-a/a Hoxa13Hd Mcoln3Va-J/J
000019   B6C3Fe-a/a-Itpr1opt/J
001022   B6C3FeF1/J a/a
001752   B6CBCa Aw-J/A-T(7;15)9H/J
006450   B6EiC3 a/A-Vss/GrsrJ
000971   B6EiC3 a/A-Och/J
000551   B6EiC3 a/A-Tbx15de-H/J
000557   B6EiC3-+ a/LnpUl A/J
000504   B6EiC3Sn a/A-Cacnb4lh/J
000553   B6EiC3Sn a/A-Egfrwa2 Wnt3avt/J
000503   B6EiC3Sn a/A-Gy/J
001811   B6EiC3Sn a/A-Otcspf-ash/J
002343   B6EiC3Sn a/A-Otcspf/J
000391   B6EiC3Sn a/A-Pax6Sey-Dey/J
001924   B6EiC3Sn a/A-Ts(1716)65Dn
001923   B6EiC3Sn a/A-Ts(417)2Lws TimT(4;17)3Lws/J
001875   B6EiC3SnF1/J
000200   C3FeB6 A/Aw-J-Ankank/J
000638   C3FeB6 A/Aw-J-Spnb4qv-J/J
000225   C3FeLe.B6 a/a-Ptpn6me/J
008425   C3FeLe.B6-a Trl/J
000198   C3FeLe.B6-a/J
000291   C3FeLe.Cg-a/a Hm KitlSl Krt71Ca-J/J
001272   C3H/HeSnJ-Ahvy/J
000099   C3HeB/FeJ-Avy/J
001886   C3HeB/FeJLe a/a-gnd/J
000584   C57BL/6J-+ T(1;2)5Ca/a +/J
000258   C57BL/6J-Ai/a/J
000774   C57BL/6J-Asy/a/J
000055   C57BL/6J-at-33J/J
000070   C57BL/6J-atd/J
000284   CWD/LeJ
000670   DBA/1J
000671   DBA/2J
001057   HPT/LeJ
000260   JGBF/LeJ
002468   KK.Cg-Ay/J
000262   LS/LeJ
000283   LT.CAST-A/J
000265   MY/HuLeJ
000308   SSL/LeJ
001759   STOCK A Tyrc Sha/J
001427   STOCK Aw us/J
000994   STOCK a Myo5ad Mregdsu/J
000064   STOCK a Tyrp1b Sisi/J
002238   STOCK a Tyrp1b shmy/J
001433   STOCK a skt/J
000579   STOCK a tp/J
000319   STOCK a us/J
002648   STOCK a/a Cln6nclf/J
000317   STOCK a/a Egfrwa2/J
000302   STOCK a/a MitfMi-wh +/+ Itpr1opt/J
000286   STOCK a/a Myo5ad fd/+ +/J
000206   STOCK a/a Tyrc-h/J
001432   STOCK a/a Tyrp1b sks/Tyrp1b +/J
000281   STOCK a/a ma Flgft/ma Flgft/J
000312   STOCK stb + a/+ Fignfi a/J
000596   STOCK T(2;11)30H/+ x AEJ-a Gdf5bp-H/J or A/J-a Gdf5bp-J/J
000970   STOCK T(2;16)28H A/T(2;16)28H a/J
000590   STOCK T(2;4)1Sn a/J
000594   STOCK T(2;8)26H a/T(2;8)26H a Tyrp1+/Tyrp1b/J
000623   TR/DiEiJ
View Strains carrying other alleles of a     (154 strains)

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms
      assigned by genotype

Kcnj6wv/Kcnj6+

        B6CBACa Aw-J/A-Kcnj6wv/J
  • nervous system phenotype
  • abnormal cerebellar Purkinje cell layer (MGI Ref ID J:72069)
    • disruptions of Purkinje cell alignment are observed
    • in more rostral regions of the central zone, Purkinje cells are ecctopic and have stunted and disorganized dendrites
    • abnormal Purkinje cell morphology (MGI Ref ID J:72069)
      • in the central zone, Purkinje cells located farther from the pial surface are clumped together in clusters
      • abnormal Purkinje cell dendrite morphology (MGI Ref ID J:72069)
        • in more rostral regions of the central zone, cells have stunted and have randomly organized dendrites
    • delaminated Purkinje cell layer (MGI Ref ID J:72069)
      • Purkinje cell layer is irregular and several cell layers thick, especially in the anterior zone of the cerebellar cortex

Kcnj6wv/Kcnj6+

        B6CBA Aw-J/A-Kcnj6wv
  • reproductive system phenotype
  • abnormal male germ cell morphology (MGI Ref ID J:23163)
    • in numerous cases, fragments of a few flagella are seen within the same cytoplasmic matrix
    • abnormal spermatid morphology (MGI Ref ID J:23163)
      • many step 2-3 spermatids often have 2 to 3 acrosomal vesicles associated with one nucleus
      • mature spermatids are often absent in affected areas of the seminiferous tubules; multinucleated spermatids are common
      • mature spermatids are deformed, maloriented and display irregular chromosomal condensation
    • oligozoospermia (MGI Ref ID J:23163)
      • sperm count in mutants is 7.1 x 105 compared to 127.7 x 105 in wild-type
  • abnormal seminiferous epithelium morphology (MGI Ref ID J:23163)
    • cellular abnormalities of the germinal epithelium are more apparent at P140 and P211; seminiferous epithelium has prominent extracellular spaces and local depletion of the epithelium
  • abnormal sperm motility (MGI Ref ID J:23163)
  • abnormal spermatogenesis (MGI Ref ID J:23163)
    • abnormal spermatid morphology (MGI Ref ID J:23163)
      • many step 2-3 spermatids often have 2 to 3 acrosomal vesicles associated with one nucleus
      • mature spermatids are often absent in affected areas of the seminiferous tubules; multinucleated spermatids are common
      • mature spermatids are deformed, maloriented and display irregular chromosomal condensation
    • oligozoospermia (MGI Ref ID J:23163)
      • sperm count in mutants is 7.1 x 105 compared to 127.7 x 105 in wild-type
  • endocrine/exocrine gland phenotype
  • abnormal seminiferous epithelium morphology (MGI Ref ID J:23163)
    • cellular abnormalities of the germinal epithelium are more apparent at P140 and P211; seminiferous epithelium has prominent extracellular spaces and local depletion of the epithelium

Kcnj6wv/Kcnj6+

        involves: C57BL/6 * CBA/CaGnLe
  • nervous system phenotype
  • abnormal cerebellum morphology (MGI Ref ID J:18348)
    • in heterozygotes the cerebellum is slightly shrunken, and some folia is less well-developed than in wild-type
    • abnormal cerebellar Purkinje cell layer (MGI Ref ID J:46842)
      • heterozygotes display a disordered Purkinje cell layer
    • abnormal cerebellar granule layer (MGI Ref ID J:46842)
      • there is moderated loss of granule cells
    • small cerebellum (MGI Ref ID J:46842)
      • cerebellum is slightly smaller in heterozygotes; it is misshapen and deflated

Kcnj6wv/Kcnj6wv

        B6CBACa Aw-J/A-Kcnj6wv/J
  • growth/size phenotype
  • decreased body weight (MGI Ref ID J:72069)
    • homozygotes have decreased body weight compared to wild-type
  • behavior/neurological phenotype
  • abnormal locomotor activation (MGI Ref ID J:6717)
    • when mutants are treated with direct dopamine agonists, they display a greater increase in locomotor activity than wild-type; amphetamine, which potentiates release of endogenous dopamine stores has less effect in mutants than in wild-type
  • ataxia (MGI Ref ID J:72069)
    • homozygotes are characterized by an ataxic gait
  • nervous system phenotype
  • abnormal nervous system morphology (MGI Ref ID J:6717)
    • in mutants the dopamine system fails to form
    • abnormal cerebellar cortex morphology (MGI Ref ID J:72069)
      • in homozygotes, Purkinje cell ectopia in the nodular zone is less pronounced but the Purkinje cell layer is several layers thick
      • abnormal cerebellar Purkinje cell layer (MGI Ref ID J:72069)
        • abnormal Purkinje cell dendrite morphology (MGI Ref ID J:72069)
          • in central zone, Purkinje cells have stunted and disorganized dendrites
        • ectopic Purkinje cell (MGI Ref ID J:72069)
          • in the central zone of the cerebellar cortex, almost all Purkinje cells are clustered ectopically
    • abnormal cerebellar lobule formation (MGI Ref ID J:72069)
      • in homozygotes, normal lobation is disrupted
    • abnormal dopaminergic neuron morphology (MGI Ref ID J:6717)
      • in mutants fewer large neurons are seen in the midbrain
    • abnormal substantia nigra morphology (MGI Ref ID J:6717)
      • in mutants the pars compacta and substantia nigra appear hypocellular compared to wild-type
  • decreased dopamine level (MGI Ref ID J:6717)
    • dopamine levels are decreased by 27% in the olfactory tubercle, 77% lower in the frontal cortex and 75% lower in the striatum in 6 month-old mutants compared to wild-type
  • homeostasis/metabolism phenotype
  • *normal* homeostasis/metabolism phenotype (MGI Ref ID J:29151)
    • no aberrant bleeding time after tail vein nick

Kcnj6wv/Kcnj6wv

        B6CBA Aw-J/A-Kcnj6wv
  • reproductive system phenotype
  • abnormal male germ cell morphology (MGI Ref ID J:23163)
    • in numerous cases, fragments of a few flagella are seen within the same cytoplasmic matrix
    • abnormal spermatid morphology (MGI Ref ID J:106298)
      • spermatid nuclei often show irregular chromosomal condensation
      • numerous multinucleated spermatids are present at P21
    • globozoospermia (MGI Ref ID J:23163)
      • at P56, numerous round spermatids are swollen, multinucleated, show defects in acrosomal structures and are undergoing degeneration
    • oligozoospermia (MGI Ref ID J:23163)
      • sperm count in mutants is 7.1 x 105 compared to 202.5 x 105 in wild-type
  • abnormal seminiferous tubule morphology (MGI Ref ID J:23163)
    • in P21 mutants, there is a lack of patent lumina
    • abnormal Sertoli cell morphology (MGI Ref ID J:23163)
      • at P21, mutants show Sertoli cells with slightly vacuolated cytoplasm
      • at P56, defects in seminiferous tubule epithelium are often associated with shrinkage of the apical processes of Sertoli cells, their disconnection from the surface of germ cells and paucity of mature spermatids
      • at P56, degenerating Sertoli cells of mutants are electron-dense, conspicuously shrunken, and have a foamy cytoplasmic appearance; many are round with small nuclei that have deeply folded surface configurations
    • abnormal seminiferous epithelium morphology (MGI Ref ID J:23163)
      • at P56 in mutants seminiferous epithelium displays progressive atrophic changes and many cellular aberrations
  • abnormal sperm motility (MGI Ref ID J:23163)
    • in mutants, percentage of mobile sperm is 23.8% compared to 73% in wild-type
  • abnormal spermatogenesis (MGI Ref ID J:23163)
    • in seminiferous tubules at P21, mutants have less advanced spermatid maturation and there are numerous multinucleated spermatids
    • cytoplasmic processes are observed which incorporate parallel membranous structures that show connections to the cisternae and vesicles of the smooth endoplasmic reticulum
    • abnormal spermatid morphology (MGI Ref ID J:106298)
      • spermatid nuclei often show irregular chromosomal condensation
      • numerous multinucleated spermatids are present at P21
    • globozoospermia (MGI Ref ID J:23163)
      • at P56, numerous round spermatids are swollen, multinucleated, show defects in acrosomal structures and are undergoing degeneration
    • oligozoospermia (MGI Ref ID J:23163)
      • sperm count in mutants is 7.1 x 105 compared to 202.5 x 105 in wild-type
  • homeostasis/metabolism phenotype
  • abnormal ion homeostasis (MGI Ref ID J:106298)
    • resting Ca2+ levels are elevated in mutant cerebellar granule neurons compared to wild-type; high potassium stimulation evokes less [Ca2+] change in mutant cerebellar granule neurons than in wild-type
  • endocrine/exocrine gland phenotype
  • abnormal seminiferous tubule morphology (MGI Ref ID J:23163)
    • in P21 mutants, there is a lack of patent lumina
    • abnormal Sertoli cell morphology (MGI Ref ID J:23163)
      • at P21, mutants show Sertoli cells with slightly vacuolated cytoplasm
      • at P56, defects in seminiferous tubule epithelium are often associated with shrinkage of the apical processes of Sertoli cells, their disconnection from the surface of germ cells and paucity of mature spermatids
      • at P56, degenerating Sertoli cells of mutants are electron-dense, conspicuously shrunken, and have a foamy cytoplasmic appearance; many are round with small nuclei that have deeply folded surface configurations
    • abnormal seminiferous epithelium morphology (MGI Ref ID J:23163)
      • at P56 in mutants seminiferous epithelium displays progressive atrophic changes and many cellular aberrations

Kcnj6wv/Kcnj6wv

        involves: C57BL/6 * CBA/CaGnLe
  • behavior/neurological phenotype
  • ataxia (MGI Ref ID J:18348)
    • homozygous mutants are distinguished by ataxia at 3 weeks of age
    • at P10, homozygotes display ataxia, tremor and hindlimb weakness
  • impaired righting response (MGI Ref ID J:18348)
    • at 7 days of age, homozygotes are distinguished by difficulty in righting themselves
  • tremors (MGI Ref ID J:18348)
    • homozygous mutants are distinguished by tremors at 3 weeks of age
  • walking backwards (MGI Ref ID J:18348)
    • A 7 days of age, homozygotes display tendency to move backward
  • muscle phenotype
  • hypotonia (MGI Ref ID J:18348)
    • homozygous mutants are distinguished by hypotonia at 3 weeks of age
  • endocrine/exocrine gland phenotype
  • abnormal testis morphology (MGI Ref ID J:18348)
    • at P28, germ cells are underdeveloped or delayed; degeneration of germ cells in the semiferous epithelium is apparent and no tubules have late-step elongated spermatids and 6% of the tubules show degenerating seminiferous epithelia
    • at P35, mutant males show only a small number of germ cells that have finished the first round of spermatogenesis
    • abnormal seminiferous tubule morphology (MGI Ref ID J:18348)
      • in homozygotes, the testes have few seminiferous tubules containing elongated spermatids
      • seminiferous tubule degeneration (MGI Ref ID J:18348)
        • testes of homozygotes show degenerating tubules with degenerating germ cells
        • at P35, degeneration is much more severe than that observed at P28; degenerating germ cells are present in all mutants examined
        • homozygotes display variable germinal epithelial degeneration
  • nervous system phenotype
  • abnormal cerebellar granule layer (MGI Ref ID J:46842)
    • there is a marked depletion of granule cells in the internal granule cell layer
  • abnormal cerebellar molecular layer (MGI Ref ID J:46842)
    • there are ectopic granule cells found in the molecular layer
  • abnormal external granule cell layer morphology (MGI Ref ID J:46842)
    • numerous dying cells are seen compared to wild-type
    • apoptotic cells lay mainly along the inner margin of the layer at P7 and 14; at P21 and 28, most apoptotic cells are confined to the thin external granule layer
    • at P7, the normal organization of granule cells into a proliferative and a nonprolifeative zone is not maintained in homozygotes
  • small cerebellum (MGI Ref ID J:18348)
    • homozygotes have and abnormally small and malformed cerebellum
    • at P7, the cerebellum of homozygotes is one-fifth the size of wild-type with a pronounced midline sulcus
  • reproductive system phenotype
  • abnormal epididymis morphology (MGI Ref ID J:18348)
    • lumens of epididymes of mutants are either empty or packed with degenerating germ cells with pyknotic nuclei and cellular debris; there are no long sickle-shaped nuclei indicative of mature sperm
  • abnormal testis morphology (MGI Ref ID J:18348)
    • at P28, germ cells are underdeveloped or delayed; degeneration of germ cells in the semiferous epithelium is apparent and no tubules have late-step elongated spermatids and 6% of the tubules show degenerating seminiferous epithelia
    • at P35, mutant males show only a small number of germ cells that have finished the first round of spermatogenesis
    • abnormal seminiferous tubule morphology (MGI Ref ID J:18348)
      • in homozygotes, the testes have few seminiferous tubules containing elongated spermatids
      • seminiferous tubule degeneration (MGI Ref ID J:18348)
        • testes of homozygotes show degenerating tubules with degenerating germ cells
        • at P35, degeneration is much more severe than that observed at P28; degenerating germ cells are present in all mutants examined
        • homozygotes display variable germinal epithelial degeneration
  • azoospermia (MGI Ref ID J:18348)
    • no sperm are found in seminiferous tubules of male homozygotes
  • male infertility (MGI Ref ID J:18348)
  • cellular phenotype
  • increased apoptosis (MGI Ref ID J:46842)
    • in homozygotes there are many more apoptotic germ cells than in wild-type
    • at P7 and 14, there is much greater cell death in the cerebellum of homozygotes

Kcnj6wv/Kcnj6wv

        involves: C57BL/6J * CBA
  • nervous system phenotype
  • abnormal hippocampal mossy fiber morphology (MGI Ref ID J:30108)
    • mossy fibers emanating from the hilus of the dentate gyrus are not confined to the layers flanking the pyramidal cell layer
  • abnormal hippocampus pyramidal cell layer (MGI Ref ID J:30108)
    • in homozygous mutants the pyramidal cell layer of area CA3 appears to be thicker than normal
    • subdivision of the pyramidal layer into 2 and 3 layers is seen in area CA3
  • ectopic pyramidal neurons (MGI Ref ID J:30108)
    • ectopic pyramidal cells are seen in the stratum radiatum and/or in the stratum oriens: these appear as small clusters or single cells surrounded by neuropil

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

Kcnj6wv/Kcnj6+

        involves: C57BL/6J
  • cellular phenotype
  • abnormal cell migration (MGI Ref ID J:16025)
    • prior to P4, granule cell migration is delayed
  • increased apoptosis (MGI Ref ID J:16025)
    • more pyknotic nuclei are observed in the external granule layer of heterozygotes at all times compared to wild-type
  • nervous system phenotype
  • abnormal cerebellum morphology (MGI Ref ID J:16025)
    • cerebellar hemispheres in heterozygotes are 37% smaller than wild-type
    • at P8, total width of the cerebellum is decreased by 13%
    • abnormal cerebellar molecular layer (MGI Ref ID J:16025)
      • in heterozygotes the molecular layer is thin and relative acellular at P4; at P6 the molecular layer appears to be hypercellular compared to wild-type
      • in the cerebellar hemispheres, 40% fewer pyknotic nuclei are observed than in the vermis
    • abnormal vermis morphology (MGI Ref ID J:16025)
      • from P2 to P4, area of the vermis is 33% smaller in heterozygotes than in wild-type; by P8, the vermal area of mutants is 57% of the area of wild-type
  • abnormal substantia nigra morphology (MGI Ref ID J:38206)
    • substantial cell loss in the substantia nigra pars compacta of the midbrain
  • behavior/neurological phenotype
  • abnormal locomotor activity (MGI Ref ID J:38206)
  • reproductive system phenotype
  • male infertility (MGI Ref ID J:38206)

Kcnj6wv/Kcnj6wv

        C57BL/6J-Kcnj6wv
  • behavior/neurological phenotype
  • abnormal motor capabilities/coordination/movement (MGI Ref ID J:13422)
    • author states that behavior resembles that of Relnrl and Rorasg; no data is given
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:13422)
    • most homozygous mutants do not live to maturity
  • nervous system phenotype
  • abnormal granule neuron (MGI Ref ID J:15542)
    • granule cell neurons in the cerebellum have an unusually large number of coated vesicles throughout the length of the neurite in mutants
    • microtubules in mutant granule cell neurites are less densely packed, curving and crossing along the neurite length and delimiting the neurite perimeter while in wild-type microtubules fill the internal dimensions of the neurite
    • cytoskeletal organization of mutant granule cell neurons in mutants shows a denser cytoplasmic matrix, indistinct skeletal structure of the cytoplasm, and microtubules delineating the boundary of microspike-like projections and entering them

Kcnj6wv/Kcnj6wv

        involves: C57BL/6J
  • cellular phenotype
  • abnormal cell migration (MGI Ref ID J:16025)
    • prior to P4, granule cell migration is delayed
  • increased apoptosis (MGI Ref ID J:16025)
    • percentage of pyknotic nuclei in the granule layer in cerebella of homozygotes is dramatically increased compared to wild-type and heterozygotes; this is increased in the vermis relative to that of the cerebellar hemisphere
  • nervous system phenotype
  • abnormal cerebellum morphology (MGI Ref ID J:5315)
    • cytoarchitecture of cerebellum is completely disorganized
    • there is no clear distinction between cortical layers
    • at P8, the area of the cerebellar hemispheres is reduced by 23% compared to wild-type
    • at P8 the width of the cerebellum is reduced by 10 and 13% compared to heterozygotes and wild-type respectively
    • abnormal cerebellar cortex morphology (MGI Ref ID J:5315)
      • small caliber parallel fibers are virtually absent
      • abnormal cerebellar Purkinje cell layer (MGI Ref ID J:5315)
        • Purkinje cells display disordered arrangement
        • abnormal Purkinje cell morphology (MGI Ref ID J:5315)
          • Purkinje cells display disordered arrangement
      • abnormal cerebellar granule layer (MGI Ref ID J:5315)
        • there is a severe paucity of granule cells in homozygotes
        • by P2 there is a decrease in the number of cells in the vermal external granule layer in homozygotes; the difference is mor striking at P4 and unlike wild-type or heterozygous mice, no spindle-shaped cells are observed
      • abnormal cerebellar molecular layer (MGI Ref ID J:16025)
        • the molecular layer is thin and relatively acellular from P0 to P6 compared to wild-type
    • abnormal vermis morphology (MGI Ref ID J:16025)
      • area of the vermis in homozygotes at P2 is 46% that of wild-type and 69% that of heterozygous mice; at P4, the vermis is 48% the size of wild-type

Kcnj6wv/Kcnj6wv

        involves: C57BL/6
  • nervous system phenotype
  • abnormal CNS synaptic transmission (MGI Ref ID J:35792)
  • abnormal granule neuron (MGI Ref ID J:35792)
    • in cultured cerebellar granule cells, very little inwardly rectifying K+ current can be evoked with somatostatin or by direct activation of G proteins, compared to wild-type cells

Kcnj6wv/Kcnj6wv

        C57BL/6J
  • lethality-postnatal
  • lethality at weaning (MGI Ref ID J:30520)
    • majority die at about 3 weeks of age
  • postnatal lethality (MGI Ref ID J:30520)
  • behavior/neurological phenotype
  • abnormal gait (MGI Ref ID J:30520)
  • abnormal maternal nurturing (MGI Ref ID J:30520)
  • abnormal motor coordination/ balance (MGI Ref ID J:30520)
    • coordination is better in the second week of life than in the third week
    • adult mice are unable to stand or step forward without falling over
    • impaired righting response (MGI Ref ID J:30520)
  • decreased eating behavior (MGI Ref ID J:30520)
    • mutants nurse poorly
  • induced hyperactivity (MGI Ref ID J:30520)
    • unique to cerebellar mutants: mice may leap when excited or agitated despite being neurologically compromised
  • tonic-clonic seizures (MGI Ref ID J:30520)
  • tremors (MGI Ref ID J:30520)
    • a fine, rapid tremor is seen in the trunk and extremities
    • the mouse moving accentuates the tremor
  • weaving (MGI Ref ID J:30520)
  • growth/size phenotype
  • decreased body size (MGI Ref ID J:30520)
    • obvious 8 to 10 days after birth
    • decreased body weight (MGI Ref ID J:30520)
      • mutants weigh about 50 percent of control sibling weight until the second month when they reach control sibling weight
  • life span-post-weaning/aging
  • decreased survivor rate (MGI Ref ID J:30520)
    • survivor rate is increased if normal foster mothers nurse pups
  • premature death (MGI Ref ID J:30520)
    • almost all mutants die within a year, whereas normal littermates live considerably longer
  • nervous system phenotype
  • abnormal cerebellar Purkinje cell layer (MGI Ref ID J:30520)
    • during greatest mitotic activity, some granule cells are degenerating
    • increased Purkinje cell number (MGI Ref ID J:30520)
      • relative to the volume of cerebellar cortical tissue
  • abnormal cerebellar granule layer (MGI Ref ID J:30520)
    • almost complete absence of granule cells
    • cells are restricted to the most lateral portions of the cerebellar hemisphere and paraflocculus
  • abnormal cerebellar molecular layer (MGI Ref ID J:30520)
    • during greatest mitotic activity, the majority of granule cells are degenerating
  • abnormal cerebellum development (MGI Ref ID J:30520)
    • abnormal external granule cell layer morphology (MGI Ref ID J:30520)
      • extensive degeneration of external granule cells prior to migration to form granule layer
    • absent cerebellar lobules (MGI Ref ID J:30520)
  • absent cerebellum fissure (MGI Ref ID J:30520)
  • small cerebellum (MGI Ref ID J:30520)
    • abnormally small at 5 to 7 days and thereafter
    • about one-fourth the size of normal in mature mice
  • tonic-clonic seizures (MGI Ref ID J:30520)
  • reproductive system phenotype
  • reduced female fertility (MGI Ref ID J:30520)
  • reduced male fertility (MGI Ref ID J:30520)
View Research Applications

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

Kcnj6wv related

Cell Biology Research
Channel and Transporter Defects
      potassium

Neurobiology Research
Ataxia (Movement) Defects
Cerebellar Defects
Channel and Transporter Defects
      potassium
Epilepsy

Genes & Alleles

Gene & Allele Information

 
Allele Symbol Aw-J
Allele Name white bellied agouti Jackson
Allele Type Spontaneous
Common Name(s) AWJ;
Strain of OriginC57BL/6J
Gene Symbol and Name a, nonagouti
Chromosome 2
Gene Common Name(s) AGSW; AGTI; AGTIL; ASP; As; MGC126092; MGC126093; SHEP9; agouti; agouti signal protein; agouti suppressor;
 
Allele Symbol Kcnj6wv
Allele Name weaver
Allele Type Spontaneous
Common Name(s) wv;
Strain of OriginC57BL/6J
Gene Symbol and Name Kcnj6, potassium inwardly-rectifying channel, subfamily J, member 6
Chromosome 16
Gene Common Name(s) BIR1; GIRK2; KATP2; KCNJ7; KIR3.2; MGC126596; hiGIRK2; weaver; wv;
Molecular Note A G-to-A transition at position 953 replaces a Gly with Ser at amino acid residue 156, affecting the highly conserved H5 domain of the channel. [MGI Ref ID J:29230] [MGI Ref ID J:30864]

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)

Hirano A; Dembitzer HM. 1973. Cerebellar alterations in the weaver mouse. J Cell Biol 56(2):478-86. [PubMed: 4118891]  [MGI Ref ID J:5315]

Yao W; Zhong J; Rosen CJ; Hock JM; Lee WH. 2005. Igf-I and postnatal growth of weaver mutant mice. Endocrine 26(2):117-25. [PubMed: 15888923]  [MGI Ref ID J:109832]

Additional References

Douhou A; Debeir T; Michel PP; Stankovski L; Oueghlani-Bouslama L; Verney C; Raisman-Vozari R. 2003. Differential activation of astrocytes and microglia during post-natal development of dopaminergic neuronal death in the weaver mouse. Brain Res Dev Brain Res 145(1):9-17. [PubMed: 14519489]  [MGI Ref ID J:86201]

Gao WQ; Liu XL; Hatten ME. 1992. The weaver gene encodes a nonautonomous signal for CNS neuronal differentiation. Cell 68(5):841-54. [PubMed: 1547486]  [MGI Ref ID J:2009]

Guatteo E; Fusco FR; Giacomini P; Bernardi G; Mercuri NB. 2000. The weaver mutation reverses the function of dopamine and GABA in mouse dopaminergic neurons. J Neurosci 20(16):6013-20. [PubMed: 10934250]  [MGI Ref ID J:63835]

Patil N; Cox DR; Bhat D; Faham M; Myers RM; Peterson AS. 1995. A potassium channel mutation in weaver mice implicates membrane excitability in granule cell differentiation [see comments] Nat Genet 11(2):126-9. [PubMed: 7550338]  [MGI Ref ID J:29230]

Perandones C; Costanzo RV; Kowaljow V; Pivetta OH; Carminatti H; Radrizzani M. 2004. Correlation between synaptogenesis and the PTEN phosphatase expression in dendrites during postnatal brain development. Brain Res Mol Brain Res 128(1):8-19. [PubMed: 15337313]  [MGI Ref ID J:92848]

Rotter A; Rath S; Evans JE; Frostholm A. 2000. Modulation of GABA(A) receptor subunit mRNA levels in olivocerebellar neurons of purkinje cell degeneration and weaver mutant mice. J Neurochem 74(5):2190-200. [PubMed: 10800965]  [MGI Ref ID J:61537]

Aw-J related

Aberg T; Wang XP; Kim JH; Yamashiro T; Bei M; Rice R; Ryoo HM; Thesleff I. 2004. Runx2 mediates FGF signaling from epithelium to mesenchyme during tooth morphogenesis. Dev Biol 270(1):76-93. [PubMed: 15136142]  [MGI Ref ID J:92174]

Barsh GS; Epstein CJ. 1989. Physical and genetic characterization of a 75-kilobase deletion associated with al, a recessive lethal allele at the mouse agouti locus. Genetics 121(4):811-8. [PubMed: 2566558]  [MGI Ref ID J:9799]

Baurle J; Vogten H; Grusser-Cornehls U. 1998. Course and targets of the calbindin D-28k subpopulation of primary vestibular afferents. J Comp Neurol 402(1):111-28. [PubMed: 9831049]  [MGI Ref ID J:118430]

Boran T; Lesot H; Peterka M; Peterkova R. 2005. Increased apoptosis during morphogenesis of the lower cheek teeth in tabby/EDA mice. J Dent Res 84(3):228-33. [PubMed: 15723861]  [MGI Ref ID J:112546]

Chinta SJ; Rane A; Yadava N; Andersen JK; Nicholls DG; Polster BM. 2009. Reactive oxygen species regulation by AIF- and complex I-depleted brain mitochondria. Free Radic Biol Med 46(7):939-47. [PubMed: 19280713]  [MGI Ref ID J:145908]

Cui CY; Hashimoto T; Grivennikov SI; Piao Y; Nedospasov SA; Schlessinger D. 2006. Ectodysplasin regulates the lymphotoxin-beta pathway for hair differentiation. Proc Natl Acad Sci U S A 103(24):9142-7. [PubMed: 16738056]  [MGI Ref ID J:111051]

Cui CY; Kunisada M; Esibizione D; Grivennikov SI; Piao Y; Nedospasov SA; Schlessinger D. 2007. Lymphotoxin-beta regulates periderm differentiation during embryonic skin development. Hum Mol Genet 16(21):2583-90. [PubMed: 17673451]  [MGI Ref ID J:129949]

Dickie MM. 1969. Mutations at the agouti locus in the mouse. J Hered 60(1):20-5. [PubMed: 5798139]  [MGI Ref ID J:30922]

Esibizione D; Cui CY; Schlessinger D. 2008. Candidate EDA targets revealed by expression profiling of primary keratinocytes from Tabby mutant mice. Gene 427(1-2):42-6. [PubMed: 18848976]  [MGI Ref ID J:143603]

Granholm DE; Reese RN; Granholm NH. 1996. Agouti alleles alter cysteine and glutathione concentrations in hair follicles and serum of mice (A y/a, A wJ/A wJ, and a/a). J Invest Dermatol 106(3):559-63. [PubMed: 8648194]  [MGI Ref ID J:32132]

Granholm DE; Reese RN; Granholm NH. 1995. Agouti alleles influence thiol concentrations in hair follicles and extrafollicular tissues of mice (Ay/a, AwJ/AwJ, a/a). Pigment Cell Res 8(6):302-6. [PubMed: 8789738]  [MGI Ref ID J:31403]

Jones JM; Huang JD; Mermall V; Hamilton BA; Mooseker MS; Escayg A; Copeland NG; Jenkins NA; Meisler MH. 2000. The mouse neurological mutant flailer expresses a novel hybrid gene derived by exon shuffling between Gnb5 and Myo5a. Hum Mol Genet 9(5):821-8. [PubMed: 10749990]  [MGI Ref ID J:61324]

Kappenman KE; Dvoracek MA; Harvison GA; Fuller BB; Granholm NH. 1992. Tyrosinase abundance and activity in murine hairbulb melanocytes of agouti mutants (C57BL/6J-a/a, Ay/a, and AwJ/AwJ). Pigment Cell Res Suppl 2:79-83. [PubMed: 1409442]  [MGI Ref ID J:1295]

Katoh A; Yoshida T; Himeshima Y; Mishina M; Hirano T. 2005. Defective control and adaptation of reflex eye movements in mutant mice deficient in either the glutamate receptor delta2 subunit or Purkinje cells. Eur J Neurosci 21(5):1315-26. [PubMed: 15813941]  [MGI Ref ID J:101081]

Knapp PE; Adjan VV; Hauser KF. 2009. Cell-specific loss of kappa-opioid receptors in oligodendrocytes of the dysmyelinating jimpy mouse. Neurosci Lett 451(2):114-8. [PubMed: 19110031]  [MGI Ref ID J:146365]

Lee M; Kim A; Chua SC Jr; Obici S; Wardlaw SL. 2007. Transgenic MSH overexpression attenuates the metabolic effects of a high-fat diet. Am J Physiol Endocrinol Metab 293(1):E121-31. [PubMed: 17374695]  [MGI Ref ID J:126508]

Lu W; Tsirka SE. 2002. Partial rescue of neural apoptosis in the Lurcher mutant mouse through elimination of tissue plasminogen activator. Development 129(8):2043-50. [PubMed: 11934869]  [MGI Ref ID J:111363]

Mayer TC; Fishbane JL. 1972. Mesoderm-ectoderm interaction in the production of the agouti pigmentation pattern in mice. Genetics 71(2):297-303. [PubMed: 4558326]  [MGI Ref ID J:5288]

Mitsumori K; Yasuhara K; Mori I; Hayashi S; Shimo T; Onodera H; Nomura T; Hayashi Y. 1998. Pulmonary fibrosis caused by N-methyl-N-nitrosourethane inhibits lung tumorigenesis by urethane in transgenic mice carrying the human prototype c-Ha-ras gene. Cancer Lett 129(2):181-90. [PubMed: 9719460]  [MGI Ref ID J:52138]

Monroe DG; Wipf LP; Diggins MR; Matthees DP; Granholm NH. 1998. Agouti-related maturation and tissue distribution of alpha-Melanocyte Stimulating Hormone in wild-type (AwJ/AwJ) and mutant (Ay/a,a/a) mice. Pigment Cell Res 11(5):310-3. [PubMed: 9877102]  [MGI Ref ID J:52183]

Mullen RJ. 1974. A<w-J> - white-bellied agouti-J Mouse News Lett 50:38.  [MGI Ref ID J:64104]

Mustonen T; Ilmonen M; Pummila M; Kangas AT; Laurikkala J; Jaatinen R; Pispa J; Gaide O; Schneider P; Thesleff I; Mikkola ML. 2004. Ectodysplasin A1 promotes placodal cell fate during early morphogenesis of ectodermal appendages. Development 131(20):4907-19. [PubMed: 15371307]  [MGI Ref ID J:128256]

O'donnell SM; Hansberger MW; Connolly JL; Chappell JD; Watson MJ; Pierce JM; Wetzel JD; Han W; Barton ES; Forrest JC; Valyi-Nagy T; Yull FE; Blackwell TS; Rottman JN; Sherry B; Dermody TS. 2005. Organ-specific roles for transcription factor NF-kappaB in reovirus-induced apoptosis and disease. J Clin Invest 115(9):2341-2350. [PubMed: 16100570]  [MGI Ref ID J:100906]

Peng J; Wu Z; Wu Y; Hsu M; Stevenson FF; Boonplueang R; Roffler-Tarlov SK; Andersen JK. 2002. Inhibition of caspases protects cerebellar granule cells of the weaver mouse from apoptosis and improves behavioral phenotype. J Biol Chem 277(46):44285-91. [PubMed: 12221097]  [MGI Ref ID J:119427]

Peng J; Xie L; Stevenson FF; Melov S; Di Monte DA; Andersen JK. 2006. Nigrostriatal dopaminergic neurodegeneration in the weaver mouse is mediated via neuroinflammation and alleviated by minocycline administration. J Neurosci 26(45):11644-51. [PubMed: 17093086]  [MGI Ref ID J:114943]

Poole TW. 1975. Dermal-epidermal interactions and the action of alleles at the agouti locus in the mouse. Dev Biol 42(2):203-10. [PubMed: 1090472]  [MGI Ref ID J:5519]

Probst FJ; Cooper ML; Cheung SW; Justice MJ. 2008. Genotype, phenotype, and karyotype correlation in the XO mouse model of Turner Syndrome. J Hered 99(5):512-7. [PubMed: 18499648]  [MGI Ref ID J:138994]

Smith DE; Xu SG. 2003. Ultrastructural organization of GABA-like immunoreactive profiles in the weaver substantia nigra. J Neurocytol 32(3):293-303. [PubMed: 14724391]  [MGI Ref ID J:121345]

Vandenput L; Swinnen JV; Boonen S; Van Herck E; Erben RG; Bouillon R; Vanderschueren D. 2004. Role of the androgen receptor in skeletal homeostasis: the androgen-resistant testicular feminized male mouse model. J Bone Miner Res 19(9):1462-70. [PubMed: 15312246]  [MGI Ref ID J:111491]

Wu Q; Miller RH; Ransohoff RM; Robinson S; Bu J; Nishiyama A. 2000. Elevated levels of the chemokine GRO-1 correlate with elevated oligodendrocyte progenitor proliferation in the jimpy mutant. J Neurosci 20(7):2609-17. [PubMed: 10729341]  [MGI Ref ID J:109469]

Yamago G; Takata Y; Furuta I; Urase K; Momoi T; Huh N. 2001. Suppression of hair follicle development inhibits induction of sonic hedgehog, patched, and patched-2 in hair germs in mice. Arch Dermatol Res 293(9):435-41. [PubMed: 11758785]  [MGI Ref ID J:116953]

Yoshida T; Katoh A; Ohtsuki G; Mishina M; Hirano T. 2004. Oscillating Purkinje neuron activity causing involuntary eye movement in a mutant mouse deficient in the glutamate receptor delta2 subunit. J Neurosci 24(10):2440-8. [PubMed: 15014119]  [MGI Ref ID J:97010]

van Empel VP; Bertrand AT; van der Nagel R; Kostin S; Doevendans PA; Crijns HJ; de Wit E; Sluiter W; Ackerman SL; De Windt LJ. 2005. Downregulation of apoptosis-inducing factor in harlequin mutant mice sensitizes the myocardium to oxidative stress-related cell death and pressure overload-induced decompensation. Circ Res 96(12):e92-e101. [PubMed: 15933268]  [MGI Ref ID J:110278]

Kcnj6wv related

Abbott LC; Sotelo C. 2000. Ultrastructural analysis of catecholaminergic innervation in weaver and normal mouse cerebellar cortices. J Comp Neurol 426(2):316-29. [PubMed: 10982471]  [MGI Ref ID J:120021]

Adachi K; Izumi M; Takahashi M; Mitsuma T; Oda SI. 1996. Levels of thyroid hormones in the brain of ataxic mutant mice. Med Sci Res 24(10):675-7.  [MGI Ref ID J:37975]

Adelbrecht C; Agid Y; Raisman-Vozari R. 1996. Effect of the weaver mutation on the expression of dopamine membrane transporter, tyrosine hydroxylase and vesicular monoamine transporter in dopaminergic neurons of the substantia nigra and the ventral tegmental area. Brain Res Mol Brain Res 43(1-2):291-300. [PubMed: 9037545]  [MGI Ref ID J:37661]

Adelbrecht C; Murer MG; Lauritzen I; Lesage F; Ladzunski M; Agid Y; Raisman-Vozari R. 1997. An immunocytochemical study of a G-protein-gated inward rectifier K+ channel (GIRK2) in the weaver mouse mesencephalon. Neuroreport 8(4):969-74. [PubMed: 9141074]  [MGI Ref ID J:40245]

Airaksinen MS; Thoenen H; Meyer M. 1997. Vulnerability of midbrain dopaminergic neurons in calbindin-D28k-deficient mice: lack of evidence for a neuroprotective role of endogenous calbindin in MPTP-treated and weaver mice. Eur J Neurosci 9(1):120-7. [PubMed: 9042576]  [MGI Ref ID J:40696]

Armstrong C; Hawkes R. 2001. Selective Purkinje cell ectopia in the cerebellum of the Weaver mouse. J Comp Neurol 439(2):151-61. [PubMed: 11596045]  [MGI Ref ID J:72069]

Bahjaoui-Bouhaddi M; Padilla F; Nicolet M; Cifuentes-Diaz C; Fellmann D; Mege RM. 1997. Localized deposition of M-cadherin in the glomeruli of the granular layer during the postnatal development of mouse cerebellum. (Erratum 1997;382:139) J Comp Neurol 378(2):180-95. [PubMed: 9120059]  [MGI Ref ID J:38641]

Bakalian A; Kopmels B; Messer A; Fradelizi D; Delhaye-Bouchaud N; Wollman E; Mariani J. 1992. Peripheral macrophage abnormalities in mutant mice with spinocerebellar degeneration. Res Immunol 143(1):129-39. [PubMed: 1565842]  [MGI Ref ID J:2228]

Bandmann O; Davis MB; Marsden CD; Wood NW. 1996. The human homologue of the weaver mouse gene in familial and sporadic Parkinson's disease. Neuroscience 72(4):877-9. [PubMed: 8735215]  [MGI Ref ID J:33925]

Bare DJ; Ghetti B; Richter JA. 1995. The tyrosine kinase inhibitor genistein increases endogenous dopamine release from normal and weaver mutant mouse striatal slices. J Neurochem 65(5):2096-104. [PubMed: 7595495]  [MGI Ref ID J:29348]

Baurle J; Frischmuth S; Kranda K. 2004. TRAIL-related death receptors in normal, Lurcher and weaver mutant mouse brain. Neurosci Lett 372(1-2):46-51. [PubMed: 15531086]  [MGI Ref ID J:107527]

Baurle J; Grover BG; Grusser-Cornehls U. 1992. Plasticity of GABAergic terminals in Deiters' nucleus of weaver mutant and normal mice: a quantitative light microscopic study. Brain Res 591(2):305-18. [PubMed: 1446244]  [MGI Ref ID J:2651]

Baurle J; Grusser-Cornehls U. 1994. Calbindin D-28k in the lateral vestibular nucleus of mutant mice as a tool to reveal Purkinje cell plasticity. Neurosci Lett 167(1-2):85-8. [PubMed: 8177535]  [MGI Ref ID J:19042]

Baurle J; Guldin W. 1998. Vestibular ganglion neurons survive the loss of their cerebellar targets. Neuroreport 9(18):4119-22. [PubMed: 9926858]  [MGI Ref ID J:54045]

Baurle J; Hoshi M; Grusser-Cornehls U. 1998. Dependence of parvalbumin expression on Purkinje cell input in the deep cerebellar nuclei. J Comp Neurol 392(4):499-514. [PubMed: 9514513]  [MGI Ref ID J:118391]

Baurle J; Oestreicher AB; Gispen WH; Grusser-Cornehls U. 1994. Lesion-specific pattern of immunocytochemical distribution of growth-associated protein B-50 (GAP-43) in the cerebellum of Weaver and PCD-mutant mice: lack of B-50 involvement in neuroplasticity of Purkinje cell terminals? J Neurosci Res 38(3):327-35. [PubMed: 7932867]  [MGI Ref ID J:18698]

Bayer SA; Triarhou LC; Thomas JD; Ghetti B. 1994. Correlated quantitative studies of the neostriatum, nucleus accumbens, substantia nigra, and ventral tegmental area in normal and weaver mutant mice. J Neurosci 14(11 Pt 2):6901-10. [PubMed: 7965086]  [MGI Ref ID J:21415]

Bayer SA; Wills KV; Triarhou LC; Thomas JD; Ghetti B. 1995. Systematic differences in time of dopaminergic neuron origin between normal mice and homozygous weaver mutants. Exp Brain Res 105(2):200-8. [PubMed: 7498373]  [MGI Ref ID J:29028]

Bayer SA; Wills KV; Triarhou LC; Verina T; Thomas JD; Ghetti B. 1995. Selective vulnerability of late-generated dopaminergic neurons of the substantia nigra in weaver mutant mice. Proc Natl Acad Sci U S A 92(20):9137-40. [PubMed: 7568088]  [MGI Ref ID J:29084]

Bayer SA; Wills KV; Wei J; Feng Y; Dlouhy SR; Hodes ME; Verina T; Ghetti B. 1996. Phenotypic effects of the weaver gene are evident in the embryonic cerebellum but not in the ventral midbrain. Brain Res Dev Brain Res 96(1-2):130-7. [PubMed: 8922675]  [MGI Ref ID J:36709]

Beattie CE; Kolva B; Siegel RE. 1995. GABAA receptor subunit mRNA expression in the weaver cerebellum: modulation by cell-cell interactions. Brain Res Dev Brain Res 88(2):171-7. [PubMed: 8665663]  [MGI Ref ID J:29738]

Berrebi AS; Morgan JI; Mugnaini E. 1990. The Purkinje cell class may extend beyond the cerebellum. J Neurocytol 19(5):643-54. [PubMed: 2077109]  [MGI Ref ID J:121314]

Bjerregaard A; Jorgensen OS. 1994. Ontogeny of the cell adhesion molecule L1 in the cerebellum of weaver and reeler mutant mice. Neurochem Res 19(7):789-93. [PubMed: 7969746]  [MGI Ref ID J:19752]

Bjerregaard A; Mogensen HS; Hack N; Balazs R; Jorgensen OS. 1997. Weaver mutant mouse cerebellar granule cells respond normally to chronic depolarization. Int J Dev Neurosci 15(2):155-62. [PubMed: 9178034]  [MGI Ref ID J:41142]

Blatt GJ; Eisenman LM. 1993. The olivocerebellar projection in normal (+/+), heterozygous weaver (wv/+), and homozygous weaver (wv/wv) mutant mice: comparison of terminal pattern and topographic organization. Exp Brain Res 95(2):187-201. [PubMed: 7693502]  [MGI Ref ID J:21302]

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Roffler-Tarlov S; Turey M. 1982. The content of amino acids in the developing cerebellar cortex and deep cerebellar nuclei of granule cell deficient mutant mice. Brain Res 247(1):65-73. [PubMed: 6127146]  [MGI Ref ID J:6875]

Rossi P; De Filippi G; Armano S; Taglietti V; D'Angelo E. 1998. The weaver mutation causes a loss of inward rectifier current regulation in premigratory granule cells of the mouse cerebellum. J Neurosci 18(10):3537-47. [PubMed: 9570785]  [MGI Ref ID J:47454]

Rotter A; Rath S; Evans JE; Frostholm A. 2000. Modulation of GABA(A) receptor subunit mRNA levels in olivocerebellar neurons of purkinje cell degeneration and weaver mutant mice. J Neurochem 74(5):2190-200. [PubMed: 10800965]  [MGI Ref ID J:61537]

Ryo Y; Miyawaki A; Furuichi T; Mikoshiba K. 1993. Expression of the metabotropic glutamate receptor mGluR1 alpha and the ionotropic glutamate receptor GluR1 in the brain during the postnatal development of normal mouse and in the cerebellum from mutant mice. J Neurosci Res 36(1):19-32. [PubMed: 8230318]  [MGI Ref ID J:14425]

Salinas PC; Fletcher C; Copeland NG; Jenkins NA; Nusse R. 1994. Maintenance of Wnt-3 expression in Purkinje cells of the mouse cerebellum depends on interactions with granule cells. Development 120(5):1277-86. [PubMed: 8026336]  [MGI Ref ID J:18125]

Savy C; Martin-Martinelli E; Simon A; Duyckaerts C; Verney C; Adelbrecht C; Raisman-Vozari R; Nguyen-Legros J. 1999. Altered development of dopaminergic cells in the retina of weaver mice. J Comp Neurol 412(4):656-68. [PubMed: 10464361]  [MGI Ref ID J:57030]

Schein JC; Hunter DD; Roffler-Tarlov S. 1998. Girk2 expression in the ventral midbrain, cerebellum, and olfactory bulb and its relationship to the murine mutation weaver. Dev Biol 204(2):432-50. [PubMed: 9882481]  [MGI Ref ID J:51929]

Schmidt MJ; Sawyer BD; Perry KW; Fuller RW; Foreman MM; Ghetti B. 1982. Dopamine deficiency in the weaver mutant mouse. J Neurosci 2(3):376-80. [PubMed: 7062116]  [MGI Ref ID J:6717]

Schneider JS; Smith MG; DiStefano L; Berrian J. 1994. GM1 ganglioside treatment partially reverses the nigrostriatal dopamine defect in the weaver mutant mouse. Brain Res 636(2):353-6. [PubMed: 7912161]  [MGI Ref ID J:16748]

Schwartz NB; Szabo M; Verina T; Wei J; Dlouhy SR; Won L; Heller A ; Hodes ME ; Ghetti B. 1998. Hypothalamic-pituitary-gonadal axis in the mutant weaver mouse. Neuroendocrinology 68(6):374-85. [PubMed: 9873201]  [MGI Ref ID J:51910]

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Shankar H; Kahner BN; Prabhakar J; Lakhani P; Kim S; Kunapuli SP. 2006. G-protein-gated inwardly rectifying potassium channels regulate ADP-induced cPLA2 activity in platelets through Src family kinases. Blood 108(9):3027-34. [PubMed: 16857990]  [MGI Ref ID J:140451]

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Simon A; Savy C; Martin-Martinelli E; Douhou A; Frederic F; Verney C; Nguyen-Legros J; Raisman-Vozari R. 2000. Paradoxical increase of tyrosine hydroxylase-immunoreactive retinopetal fibers in the weaver mouse. Brain Res Dev Brain Res 121(1):113-7. [PubMed: 10837899]  [MGI Ref ID J:109195]

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Slesinger PA; Stoffel M; Jan YN; Jan LY. 1997. Defective gamma-aminobutyric acid type B receptor-activated inwardly rectifying K+ currents in cerebellar granule cells isolated from weaver and Girk2 null mutant mice. Proc Natl Acad Sci U S A 94(22):12210-7. [PubMed: 9342388]  [MGI Ref ID J:43706]

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Smith DE; Xu SG. 2003. Ultrastructural organization of GABA-like immunoreactive profiles in the weaver substantia nigra. J Neurocytol 32(3):293-303. [PubMed: 14724391]  [MGI Ref ID J:121345]

Sola C; Mengod G; Ghetti B; Palacios JM; Triarhou LC. 1993. Regional distribution of the alternatively spliced isoforms of beta APP RNA transcript in the brain of normal, heterozygous and homozygous weaver mutant mice as revealed by in situ hybridization histochemistry. Brain Res Mol Brain Res 17(3-4):340-6. [PubMed: 8510506]  [MGI Ref ID J:4115]

Sola C; Mengod G; Low WC; Norton J; Ghetti B; Palacios JM; Triarhou LC. 1993. Regional distribution of amyloid beta-protein precursor, growth-associated phosphoprotein-43 and microtubule-associated protein 2 messenger RNAs in the nigrostriatal system of normal and Weaver mutant mice and effects of ventral mesencephalic grafts. Eur J Neurosci 5(11):1442-54. [PubMed: 8287193]  [MGI Ref ID J:16255]

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Sotelo C. 1990. Cerebellar synaptogenesis: what we can learn from mutant mice. J Exp Biol 153:225-49. [PubMed: 2280222]  [MGI Ref ID J:10974]

Stasi K; Mitsacos A; Giompres P; Kouvelas ED; Triarhou LC. 1999. Partial restoration of striatal GABAA receptor balance by functional mesencephalic dopaminergic grafts in mice with hereditary parkinsonism. Exp Neurol 157(2):259-67. [PubMed: 10364438]  [MGI Ref ID J:55410]

Stotz EH; Palacios JM; Landwehrmeyer B; Norton J; Ghetti B; Simon JR; Triarhou LC. 1994. Alterations in dopamine and serotonin uptake systems in the striatum of the weaver mutant mouse. J Neural Transm Gen Sect 97(1):51-64. [PubMed: 7888149]  [MGI Ref ID J:25009]

Stotz EH; Triarhou LC; Ghetti B; Simon JR. 1993. Serotonin content is elevated in the dopamine deficient striatum of the weaver mutant mouse. Brain Res 606(2):267-72. [PubMed: 8490719]  [MGI Ref ID J:4281]

Stotz-Potter EH; Ghetti B; Simon JR. 1995. Endogenous serotonin release from the dopamine-deficient striatum of the weaver mutant mouse. Neurochem Res 20(7):821-6. [PubMed: 7477675]  [MGI Ref ID J:28667]

Strazielle C; Deiss V; Naudon L; Raisman-Vozari R; Lalonde R. 2006. Regional brain variations of cytochrome oxidase activity and motor coordination in Girk2(Wv) (Weaver) mutant mice. Neuroscience 142(2):437-449. [PubMed: 16844307]  [MGI Ref ID J:113165]

Strazielle C; Lalonde R; Amdiss F; Botez MI; Hebert C; Reader TA. 1998. Distribution of dopamine transporters in basal ganglia of cerebellar ataxic mice by [125I] RTI-121 quantitative autoradiography. Neurochem Int 32(1):61-8. [PubMed: 9460703]  [MGI Ref ID J:47901]

Surmeier DJ; Mermelstein PG; Goldowitz D. 1996. The weaver mutation of GIRK2 results in a loss of inwardly rectifying K+ current in cerebellar granule cells [see comments] Proc Natl Acad Sci U S A 93(20):11191-5. [PubMed: 8855331]  [MGI Ref ID J:35792]

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Takayama C; Nakagawa S; Watanabe M; Kurihara H; Mishina M; Inoue Y. 1997. Altered intracellular localization of the glutamate receptor channel delta 2 subunit in weaver and reeler Purkinje cells. Brain Res 745(1-2):231-42. [PubMed: 9037414]  [MGI Ref ID J:40856]

Takeda H; Yoshiki A; Nishikawa S; Nishikawa S; Kunisada T; Sakakura T; Amanuma H; Kusakabe M. 1992. Expression of c-kit, a proto-oncogene of the murine W locus, in cerebella of normal and neurological mutant mice: immunohistochemical and in situ hybridization analysis. Differentiation 51(2):121-7. [PubMed: 1282111]  [MGI Ref ID J:2865]

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Triarhou LC; Sola C; Mengod G; Garcia-Ladona FJ; Landwehrmeyer B; Ghetti B; Palacios JM. 1995. Ventral mesencephalic grafts in the neostriatum of the weaver mutant mouse: structural molecule and receptor studies. Cell Transplant 4(1):39-48. [PubMed: 7728332]  [MGI Ref ID J:23210]

Triarhou LC; Sola C; Palacios JM; Mengod G. 1998. MAP2 and GAP-43 expression in normal and weaver mouse cerebellum: correlative immunohistochemical and in situ hybridization studies. Arch Histol Cytol 61(3):233-42. [PubMed: 9756100]  [MGI Ref ID J:49935]

Triarhou LC; Stotz EH; Low WC; Norton J; Ghetti B; Landwehrmeyer B; Palacios JM; Simon JR. 1994. Studies on the striatal dopamine uptake system of weaver mutant mice and effects of ventral mesencephalic grafts. Neurochem Res 19(11):1349-58. [PubMed: 7898605]  [MGI Ref ID J:21868]

Varecka L; Wu CH; Rotter A; Frostholm A. 1994. GABAA/benzodiazepine receptor alpha 6 subunit mRNA in granule cells of the cerebellar cortex and cochlear nuclei: expression in developing and mutant mice. J Comp Neurol 339(3):341-52. [PubMed: 8132866]  [MGI Ref ID J:16213]

Verina T; Norton JA; Sorbel JJ; Triarhou LC; Laferty D; Richter JA ; Simon JR ; Ghetti B. 1997. Atrophy and loss of dopaminergic mesencephalic neurons in heterozygous weaver mice. Exp Brain Res 113(1):5-12. [PubMed: 9028770]  [MGI Ref ID J:40681]

Verina T; Tang X; Fitzpatrick L; Norton J; Vogelweid C; Ghetti B. 1995. Degeneration of Sertoli and spermatogenic cells in homozygous and heterozygous weaver mice. J Neurogenet 9(4):251-65. [PubMed: 7760215]  [MGI Ref ID J:23163]

Verney C; Febvret-Muzerelle A; Gaspar P. 1995. Early postnatal changes of the dopaminergic mesencephalic neurons in the weaver mutant mouse. Brain Res Dev Brain Res 89(1):115-9. [PubMed: 8575083]  [MGI Ref ID J:30109]

Vogelweid CM; Verina T; Norton J; Harruff R; Ghetti B. 1993. Hypospermatogenesis is the cause of infertility in the male weaver mutant mouse. J Neurogenet 9(2):89-104. [PubMed: 8126599]  [MGI Ref ID J:16319]

Wei J; Dlouhy SR; Bayer S; Piva R; Verina T; Wang Y; Feng Y ; Dupree B ; Hodes ME ; Ghetti B. 1997. In situ hybridization analysis of Girk2 expression in the developing central nervous system in normal and weaver mice. J Neuropathol Exp Neurol 56(7):762-71. [PubMed: 9210872]  [MGI Ref ID J:41890]

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Health & husbandry

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

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Pricing

Pricing for USA, Canada and Mexico shipping destinations View International pricing
Price (US dollars $)
Cryorecovery Fee $1900.00
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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 View USA Canada and Mexico pricing
Price (US dollars $)
Cryorecovery Fee $2470.00
Animals Provided

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.

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Standard SupplyCryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
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  • Cryorecovery - Standard.
    We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 13 and 16 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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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    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

  • This strain is included in the Mouse Mutant Resource collection.
  • Genomic DNA is available for this strain from the Mouse DNA Resource.

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  USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
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