Strain Name:

B6.C3(Cg)-Rorasg/J

Stock Number:

002651

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Availability:

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The cerebellar cortex of homozygous mutant staggerer (Rorasg) mice show a deficiency of granule cells and Purkinje cells. They have a staggering gait, mild tremor, and hypotonia. These mice may be useful in studies of the composition and function of granule cells.

Description

Strain Information

Former Names B6.Cg-Rorasg/J    (Changed: 25-JUL-06 )
B6.C3-Rorasg/J    (Changed: 24-JUL-06 )
B6.C3(B10)-Rorasg/J    (Changed: 21-JUL-06 )
B6.Cg-Rorasg/J    (Changed: 20-JUL-06 )
Type Congenic; Mutant Strain;
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Mating SystemHeterozygote x +/+ sibling         (Female x Male)   13-OCT-11
Mating System+/+ sibling x Heterozygote         (Female x Male)   13-OCT-11
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain Mixed stock
GenerationNE9F75 (28-OCT-13)
Generation Definitions

Appearance
black, ataxic, tremors
Related Genotype: a/a Rorasg/Rorasg

black, unaffected
Related Genotype: a/a +/? or a/a Rorasg/+

Description
Mice homozygous for the staggerer spontaneous mutation (Rorasg) show a staggering gait, mild tremor, hypotonia, and small size. The cerebellar cortex of homozygous mutant mice is grossly underdeveloped with a deficiency of granule cells and Purkinje cells. The remaining granule cells migrate inward from the external layer prematurely and then degenerate. Purkinje cells are much delayed in postnatal differentiation and lack the dendritic spines on which synapses with the parallel fibers from the granule cells normally occur. Staggerer mutant mice have been used as a source of an agranulate cerebellum in a number of investigations of the composition and function of granule cells. Kopmels et al. have reported a hyperproduction of IL1 biological activity and mRNA from LPS stimulated spleen cells of Rorasg/Rorasg mice on the C57BL/6J background relative to wild type siblings. In addition, homozygous mice exhibit an enhanced susceptibility to LPS-induced lung inflammation, suggesting a role for Rora in LPS-induced inflammatory responses (Stapleton CM, et al., 2005).

Development
The first Rorasg/Rorasg mouse was observed in 1955 among the F2 progeny of a (BALB/cHm x C3H/HeJ)F1 female and a male of an obese(Lepob) stock of mixed background. The mutation was maintained for several generations by an intercross-backcross (within the same or a parallel lineage) mating scheme, then was backcrossed onto C57BL/6J for four generations. A Rorasg/+ male from the fourth backcross was mated to a female C57BL/10-Myo5ad Bmp5se mouse to introduce the dilute and short-ear mutations into the stock in repulsion with Rora6 sg ; this allowed the heterozygotes (Rorasg + +/+ Myo5ad Bmp5se to be identified by a lack of either recessive phenotype. Brother-sister inbreeding was continued. At F55, Myo5ad Bmpse was backcrossed onto C57BL/6J seven times; at F58, Rorasg was backcrossed onto C57BL/6J four times. The Rorasg + +/+ Myo5ad Bmp5se stock was then reconstructed by crossing mice of the two independent stocks and maintained by brother-sister inbreeding. In early 1997, Myo5ad and Bmp5se were bred out of the stock by selective breeding assisted by PCR testing. Based on the alleles carried by this strain for 16 SSLPs mapping within 1 cM of Rora, it appears that the mutation occurred on a C3H chromosome (Hamilton et al. 1996).

Control Information

  Control
   See control note: Wild type mice from the colony are the preferred controls. Untyped mice from the colony may be used; however, heterozygotes exhibit "accelerated loss of Purkinje cells, granule cells, and inferior olivary neurons with age" (MLC; Zanjani et al.1992; Hadj-Sahraoui et al.1997).
   Wild-type from the colony
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Rorasg allele
000285   B6.Cg-Rorasg + +/+ Myo5ad Bmp5se/J
000237   B6C3Fe a/a-Rorasg/J
View Strains carrying   Rorasg     (2 strains)

Strains carrying other alleles of Rora
005047   C57BL/6J-Rorasg-3J/J
View Strains carrying other alleles of Rora     (1 strain)

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Rorasg/Rora+

        involves: C57BL/6J
  • behavior/neurological phenotype
  • abnormal spatial learning
    • mice exhibit impaired spatial learning in a Z-maze filled with water compared with wild-type mice   (MGI Ref ID J:30686)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Rorasg/Rora+

        involves: C57BL
  • nervous system phenotype
  • abnormal cerebellum morphology   (MGI Ref ID J:1431)
    • abnormal cerebellar layer morphology   (MGI Ref ID J:1431)
      • abnormal cerebellar granule layer morphology
        • granule cell layer decreases with age   (MGI Ref ID J:1431)
        • cell density is reduced   (MGI Ref ID J:1431)
        • decreased cerebellar granule cell number
          • cell numbers are about 35% of controls   (MGI Ref ID J:1431)
      • decreased Purkinje cell number
        • significant loss of Purkinje cells at 1 year of age   (MGI Ref ID J:1431)
    • small cerebellum
      • decreased cross-sectional area of the cerebellum at 1 year of age   (MGI Ref ID J:1431)
  • abnormal inferior olivary complex morphology
    • neuron counts are 60% of controls at 1 year of age   (MGI Ref ID J:1431)

Rorasg/Rorasg

        involves: obese stock
  • mortality/aging
  • partial postnatal lethality
    • about 50% of mutants die by weaning   (MGI Ref ID J:13140)
  • nervous system phenotype
  • abnormal brain morphology   (MGI Ref ID J:13140)
    • abnormal cerebellum morphology
      • cerebellum of adults shows tiny folia with indistinct fissures   (MGI Ref ID J:13140)
      • cerebellum is less than one-third the size of wild-type littermates   (MGI Ref ID J:13140)
      • adult cerebellar cell surface with embryonic characteristics   (MGI Ref ID J:6068)
      • cells agglutinable with wheat germ agglutinin but not wit h Con-A   (MGI Ref ID J:6068)
      • abnormal cerebellar foliation   (MGI Ref ID J:13140)
      • abnormal cerebellar layer morphology   (MGI Ref ID J:5304)
        • abnormal Purkinje cell morphology
          • cells are randomly oriented   (MGI Ref ID J:121314)
          • poorly elaborated dendritic trees   (MGI Ref ID J:121314)
        • abnormal cerebellar granule layer morphology
          • granular cell layer displays a paucity of cells in mutants   (MGI Ref ID J:13140)
          • abnormal cerebellar granule cell morphology
            • external granule layer less developed at birth   (MGI Ref ID J:5304)
            • reduced rate of cell proliferation at 1 and 5 days of age   (MGI Ref ID J:5304)
            • cells migrate prematurely from the external granule layer having undergone fewer cell divisions   (MGI Ref ID J:5304)
            • conspicuous differences in the internal granule layer at 10 days of age   (MGI Ref ID J:5304)
            • decreased cerebellar granule cell number
              • 25% reduction in granule cells in the internal granule cell layer   (MGI Ref ID J:5304)
        • abnormal cerebellar molecular layer   (MGI Ref ID J:13140)
          • thin cerebellar molecular layer   (MGI Ref ID J:13140)
      • small cerebellum   (MGI Ref ID J:13140)
        • weight is significantly less than controls even before neurological symptoms appear   (MGI Ref ID J:5304)
        • less than half of control weight at 5 days of age   (MGI Ref ID J:5304)
    • abnormal cochlear VIII nucleus morphology
      • cochlear nuclei are reduced in volume and severely malformed   (MGI Ref ID J:121314)
      • dendritic spines are rare on cartwheel cells at 1 month of age   (MGI Ref ID J:121314)
      • cartwheel cells are rarely seen in adults   (MGI Ref ID J:121314)
    • abnormal olfactory bulb morphology
      • fewer astrocytes found   (MGI Ref ID J:55239)
      • dendro-dendritic asymmetrical synapses are abundant   (MGI Ref ID J:55239)
      • abnormal olfactory bulb layer morphology   (MGI Ref ID J:55239)
        • abnormal olfactory bulb external plexiform layer morphology
          • thinner than controls   (MGI Ref ID J:55239)
          • lower density of secondary dendrites   (MGI Ref ID J:55239)
          • large, empty, intercellular spaces   (MGI Ref ID J:55239)
        • abnormal olfactory bulb glomerular layer morphology
          • thinner than controls   (MGI Ref ID J:55239)
          • glomerular surface reduced about 15%   (MGI Ref ID J:55239)
          • reduced cell number and intercellular space   (MGI Ref ID J:55239)
          • reduced number of interneuronal relays   (MGI Ref ID J:55239)
        • abnormal olfactory bulb granule cell layer morphology
          • composed of several layers of anaxonal interneurons with smaller than normal cell bodies   (MGI Ref ID J:55239)
          • nuclear size is smaller than controls   (MGI Ref ID J:55239)
          • abnormal olfactory bulb granule cell morphology
            • pycnotic nuclei   (MGI Ref ID J:55239)
            • vacuolations and ruptured membranes   (MGI Ref ID J:55239)
        • abnormal olfactory bulb internal plexiform layer morphology
          • thinner than controls   (MGI Ref ID J:55239)
          • less distinct   (MGI Ref ID J:55239)
        • abnormal olfactory bulb mitral cell layer morphology
          • somewhat discontinuous   (MGI Ref ID J:55239)
        • abnormal olfactory bulb outer nerve layer morphology
          • glial cells with more processes ensheathing axons in the nerve layer   (MGI Ref ID J:55239)
      • small olfactory bulb
        • volume slightly reduced   (MGI Ref ID J:55239)
  • abnormal nervous system physiology
    • immunoreactive somatostatin is significantly elevated in both the cerebrum and in the cerebellum   (MGI Ref ID J:28478)
  • behavior/neurological phenotype
  • abnormal motor capabilities/coordination/movement   (MGI Ref ID J:13140)
    • abnormal gait
      • gait is shuffling and hesitant, interrupted every few steps by lurching motions side-to-side   (MGI Ref ID J:13140)
      • abnormal gait is apparent at ~2 weeks of age   (MGI Ref ID J:13140)
    • abnormal limb posture
      • some animals have hindlimbs held abducted and everted at 45 degrees at rest   (MGI Ref ID J:13140)
    • ataxia   (MGI Ref ID J:13140)
      • clearly visible in all mice   (MGI Ref ID J:46854)
      • mice stumble at a rate 40 times greater than WT mice   (MGI Ref ID J:46854)
      • treatment with a thyrotropin releasing hormone analog improves ataxia (fewer falls in a given distance travelled)   (MGI Ref ID J:18435)
    • decreased grip strength
      • mice have a mean hanging time of 12 seconds compared to over 3 minutes for WT mice   (MGI Ref ID J:46854)
    • hypoactivity
      • mutants remain stationary much more than littermates   (MGI Ref ID J:13140)
    • impaired coordination   (MGI Ref ID J:13140)
      • mice fall off an elevated rod on average of 13 seconds compared to over 3 minutes for WT mice   (MGI Ref ID J:46854)
    • limb grasping
      • is observed in all mice   (MGI Ref ID J:46854)
    • tremors
      • mild tremors accompany initiation of movement   (MGI Ref ID J:13140)
  • abnormal motor learning
    • mice have an impaired ability to learn how to hang onto a rotating rod   (MGI Ref ID J:46854)
    • mice hang onto the rod as opposed the walking strategy WT mice exclusively use   (MGI Ref ID J:46854)
    • scores do not improve with 10 days of training   (MGI Ref ID J:46854)
  • growth/size/body phenotype
  • decreased body size
    • mutants are smaller than littermates   (MGI Ref ID J:13140)
  • homeostasis/metabolism phenotype
  • abnormal hormone level
    • prothyrotropin releasing hormone levels are elevated in the thalamus, cerebellum, brainstem, and spinal cord   (MGI Ref ID J:28467)

Rorasg/Rorasg

        involves: C57BL/6
  • homeostasis/metabolism phenotype
  • abnormal lipid homeostasis
    • plasma APOA1 and APOA2 concentrations are approximately 2 fold lower than in wild-type controls on a normal diet, and the Apoa1 mRNA level in intestine is diminished relative to wild-type, although liver expression of Apoa1 is comparable with wild-type   (MGI Ref ID J:52105)
    • the production rate of APOA1 is diminished, but the fractional catabolic rate is comparable to wild-type   (MGI Ref ID J:52105)
    • decreased circulating cholesterol level
      • plasma total cholesterol levels are significantly lower in both male and female homozygotes than in wild-type controls   (MGI Ref ID J:52105)
      • although cholesterol levels increase on an atherosclerotic diet, homozygotes still have lower plasma cholesterol than wild-type controls also fed this diet   (MGI Ref ID J:52105)
      • decreased circulating HDL cholesterol level
        • plasma HDL cholesterol level is significantly lower in both male and female homozygotes than in wild-type controls, and females have significantly lower plasma HDL level than males both for the homozygous and wild-type data sets. This is true even on an atherogenic diet.   (MGI Ref ID J:52105)
  • cardiovascular system phenotype
  • abnormal vasoconstriction
    • contractions of the mesenteric artery induced by phenylephrin or serotonin are less than in controls   (MGI Ref ID J:109735)
  • abnormal vasodilation
    • blood flow induced dilation of the mesenteric artery is less than for controls   (MGI Ref ID J:109735)
    • endothelium-dependent and independent dilation is reduced   (MGI Ref ID J:109735)
  • atherosclerotic lesions
    • the atherosclerotic lesions induced in homozygotes by 9 weeks of an atherosclerotic diet have a 6 fold greater area in female homozygotes and a 7.5 fold greater area in male homozygotes than those in wild-type controls on the same diet   (MGI Ref ID J:52105)
    • increased susceptibility to atherosclerosis
      • Although homozygotes fed a normal diet do not display abnormal atherosclerotic lesions, 9 weeks of an atherosclerotic diet induces exaggerated altherosclerotic lesions compared with wild-type controls on the atherosclerotic diet   (MGI Ref ID J:52105)
  • decreased mean systemic arterial blood pressure
    • 80mmHg vs 87mmHg for controls   (MGI Ref ID J:109735)
    • phenylephrine causes less blood pressure increase than in controls   (MGI Ref ID J:109735)
    • acetyl choline causes less blood pressure decrease than in controls   (MGI Ref ID J:109735)
  • increased angiogenesis
    • significantly increased in the ischemic leg 28 days after femoral artery ligature   (MGI Ref ID J:115425)
    • 80% rise in vascular density relative to controls   (MGI Ref ID J:115425)
    • 3 fold increase in capillary density relative to controls   (MGI Ref ID J:115425)
  • immune system phenotype
  • *normal* immune system phenotype
    • white blood cell, lymphocyte, and neutrophil counts are not significantly different between homozygotes and wild-type controls   (MGI Ref ID J:52105)
    • abnormal immune system organ morphology   (MGI Ref ID J:2228)
      • spleen atrophy   (MGI Ref ID J:2228)
      • thymus atrophy   (MGI Ref ID J:2228)
    • abnormal immune system physiology
      • abnormal immune responses   (MGI Ref ID J:2228)
      • abnormal macrophage physiology
        • LPS stimulated macrophage produce 2 fold more IL-1 relative to macrophage of controls   (MGI Ref ID J:28095)
      • increased IgE level
        • levels are elevated in unsensitized mice   (MGI Ref ID J:135863)
        • OVA sensitization causes an additional elevation in IgE levels   (MGI Ref ID J:135863)
        • OVA challenge of sensitized mice causes a very great increase in IgE levels   (MGI Ref ID J:135863)
      • increased interleukin-1 secretion
        • LPS stimulated macrophage produce 2 fold more IL-1 relative to macrophage of controls   (MGI Ref ID J:28095)
      • lung inflammation
        • OVA sensitized and challenged mice experience less infiltration of lymphocytes and polymorphonuclear cells into peribronchiolar and perivascular regions of the lungs   (MGI Ref ID J:135863)
        • less alveolar infiltration as well   (MGI Ref ID J:135863)
        • less increase of inflammatory cells in bronchoalveolar lavage   (MGI Ref ID J:135863)
        • less increase of IL-4, IL-5, and IL-13   (MGI Ref ID J:135863)
  • nervous system phenotype
  • abnormal cerebellum morphology
    • embryonic, high sialic acid forms of N-CAM persist on cell surfaces to 14 and 21 days of age   (MGI Ref ID J:6930)
  • abnormal inferior olivary complex morphology
    • individual olivary subnuclei are poorly defined relative to controls   (MGI Ref ID J:20982)
    • definition of subnuclei is better in 21 day old mice but regresses in adults   (MGI Ref ID J:20982)
    • olivary dendrites tend to be multipolar and well branched   (MGI Ref ID J:20982)
    • number of neurons drops 30% within 3 days of birth and continues to decline for 2 months   (MGI Ref ID J:20982)
    • number of neurons in adults is decreased 60% relative to newborns   (MGI Ref ID J:20982)
    • adult cell bodies are smaller than in newborns   (MGI Ref ID J:20982)
    • inferior olive extends further dorsally in the midline   (MGI Ref ID J:28468)
    • decreased cell density   (MGI Ref ID J:28468)
  • reproductive system phenotype
  • abnormal external female genitalia morphology
    • vaginal opening averaged 35 days (never earlier than 31 days) while controls averaged 28 days (earliest 26 days)   (MGI Ref ID J:1960)
  • abnormal female reproductive system physiology   (MGI Ref ID J:1960)
    • abnormal estrous cycle
      • irregular cycles   (MGI Ref ID J:1960)
      • delayed estrous cycle
        • onset of estrus at 45 days vs 40 days for controls   (MGI Ref ID J:1960)
      • prolonged estrus
        • prolonged vaginal estrus relative to controls   (MGI Ref ID J:1960)
      • prolonged metestrus   (MGI Ref ID J:1960)
    • reduced female fertility
      • no females mate before 2.5 months of age   (MGI Ref ID J:1960)
      • females sexual activity decreases after 4.5 months   (MGI Ref ID J:1960)
      • only one of 10 females still mating at 6 months of age   (MGI Ref ID J:1960)
  • abnormal fertility/fecundity
    • some couples receiving vestibular stimulation mated between the ages of 36 and 89 days of age   (MGI Ref ID J:14535)
    • no unstimulated couples mated   (MGI Ref ID J:14535)
    • reduced female fertility
      • no females mate before 2.5 months of age   (MGI Ref ID J:1960)
      • females sexual activity decreases after 4.5 months   (MGI Ref ID J:1960)
      • only one of 10 females still mating at 6 months of age   (MGI Ref ID J:1960)
  • abnormal male reproductive system physiology
    • vestibular stimulation improves the ability of males to mate with experienced females   (MGI Ref ID J:14535)
    • one to two months of social isolation results in increased mounting behavior when a receptive female is encountered   (MGI Ref ID J:26698)
    • successful mating occurs from 35-160 days after the end of male isolation   (MGI Ref ID J:26698)
  • behavior/neurological phenotype
  • abnormal maternal nurturing
    • females from heterozygous parents fail to nurture pups to weaning   (MGI Ref ID J:14497)
    • females from selected homozygous lineages sometimes (9/14) raise pups to weaning although growth rate of pups is lower than normal   (MGI Ref ID J:14497)
  • abnormal spatial learning
    • higher latency to escape at all time tested using two types of water maze   (MGI Ref ID J:30012)
    • less age related degradation in latency to escape although it is always greater than in controls   (MGI Ref ID J:30012)
  • respiratory system phenotype
  • abnormal lung compliance
    • lung resistance in response to methacholine challenge of OVA exposed mice fails to increase   (MGI Ref ID J:135863)
  • abnormal respiratory mucosa morphology
    • less mucous cell hyperplasia in airways after OVA exposure of sensitized mice   (MGI Ref ID J:135863)
  • lung inflammation
    • OVA sensitized and challenged mice experience less infiltration of lymphocytes and polymorphonuclear cells into peribronchiolar and perivascular regions of the lungs   (MGI Ref ID J:135863)
    • less alveolar infiltration as well   (MGI Ref ID J:135863)
    • less increase of inflammatory cells in bronchoalveolar lavage   (MGI Ref ID J:135863)
    • less increase of IL-4, IL-5, and IL-13   (MGI Ref ID J:135863)
  • taste/olfaction phenotype
  • *normal* taste/olfaction phenotype
    • male mice are able to distinguish between the vaginal secretions of estrus and non-estrus females   (MGI Ref ID J:15645)
    • abnormal olfaction
      • aversive threshold concentration for butanol is increased   (MGI Ref ID J:39053)
      • attractive threshold concentration for vanillin is increased   (MGI Ref ID J:39053)
      • amyl alchohol odor induced evoked field potential shows an increased latency preceding the functional response of mitral cells in the olfactory bulb   (MGI Ref ID J:107925)
      • N1 and N2 response amplitudes of the evoked field potential are significantly reduced   (MGI Ref ID J:107925)
  • hematopoietic system phenotype
  • abnormal macrophage physiology
    • LPS stimulated macrophage produce 2 fold more IL-1 relative to macrophage of controls   (MGI Ref ID J:28095)
  • increased IgE level
    • levels are elevated in unsensitized mice   (MGI Ref ID J:135863)
    • OVA sensitization causes an additional elevation in IgE levels   (MGI Ref ID J:135863)
    • OVA challenge of sensitized mice causes a very great increase in IgE levels   (MGI Ref ID J:135863)
  • spleen atrophy   (MGI Ref ID J:2228)
  • thymus atrophy   (MGI Ref ID J:2228)
  • muscle phenotype
  • abnormal vasoconstriction
    • contractions of the mesenteric artery induced by phenylephrin or serotonin are less than in controls   (MGI Ref ID J:109735)
  • abnormal vasodilation
    • blood flow induced dilation of the mesenteric artery is less than for controls   (MGI Ref ID J:109735)
    • endothelium-dependent and independent dilation is reduced   (MGI Ref ID J:109735)
  • endocrine/exocrine gland phenotype
  • thymus atrophy   (MGI Ref ID J:2228)

Rorasg/Rorasg

        involves: C57BL
  • nervous system phenotype
  • abnormal cerebellum morphology
    • cell surface antigen characteristics more embryonic in nature   (MGI Ref ID J:6088)
    • abnormal cerebellar layer morphology   (MGI Ref ID J:5968)
      • abnormal cerebellar Purkinje cell layer
        • Purkinje cell band thicker at 3 days of age   (MGI Ref ID J:5968)
        • abnormal Purkinje cell morphology
          • cells smaller in size with thinner processes   (MGI Ref ID J:5968)
          • larger, typical Purkinje cells are absent in intermediate regions of the cerebellum   (MGI Ref ID J:6185)
          • 60-90% of Purkinje cells are lost   (MGI Ref ID J:6185)
          • multiple climbing fiber synaptic connections to each Purkinje cell are maintained   (MGI Ref ID J:6260)
          • abnormal Purkinje cell dendrite morphology
            • no synapses form between Purkinje cell spines and the parallel fibers at 14 to 43 days of age   (MGI Ref ID J:5968)
            • reduced growth of dendrite arbor   (MGI Ref ID J:5968)
          • abnormal Purkinje cell differentiation
            • retarded   (MGI Ref ID J:5968)
        • delaminated Purkinje cell layer
          • typical laminar array fails to form   (MGI Ref ID J:5968)
      • abnormal cerebellar granule layer morphology   (MGI Ref ID J:5968)
        • abnormal cerebellar granule cell morphology
          • no synapses form between the parallel fibers and the Purkinje cell spines at 14 to 43 days of age   (MGI Ref ID J:5968)
          • synapses do develop between parallel fibers and stellate and basket cells   (MGI Ref ID J:5968)
          • degenerating parallel fibers begin to appear around 7 days and is well advanced by 14 days   (MGI Ref ID J:5968)
        • thin cerebellar granule layer   (MGI Ref ID J:5968)
      • thin cerebellar molecular layer   (MGI Ref ID J:5968)
    • abnormal cerebellum deep nucleus morphology
      • white matter in deep cerebellar nuclei is reduced to 42% of controls   (MGI Ref ID J:6554)
      • area occupied by deep neurons is reduced to 36% of controls but cell numbers are not reduced   (MGI Ref ID J:6554)
      • neurons in the deep nuclei are smaller than seen in controls   (MGI Ref ID J:6554)
    • small cerebellum
      • 62% of normal weight at birth   (MGI Ref ID J:6875)
      • weight is 36% of normal at 7 days   (MGI Ref ID J:6875)
  • abnormal neurotransmitter level
    • taurine and aspartate levels decline between 7 and 10 days   (MGI Ref ID J:6875)
    • low levels persist   (MGI Ref ID J:6875)
    • GABA levels in deep cerebellar nuclei are reduced at all ages examined   (MGI Ref ID J:6875)
    • GABA levels are 52% of controls at 7 days and 30% at 3 weeks   (MGI Ref ID J:6875)
    • abnormal synaptic glutamate release
      • glutamate levels drop between 7 and 10 days   (MGI Ref ID J:6875)
      • levels continue to drop over time   (MGI Ref ID J:6875)
  • homeostasis/metabolism phenotype
  • abnormal noradrenaline level
    • significantly elevated in cerebellum   (MGI Ref ID J:164122)
    • significantly elevated in cerebral cortex   (MGI Ref ID J:164122)
    • measurably increased in the spinal cord   (MGI Ref ID J:164122)
  • cellular phenotype
  • abnormal Purkinje cell differentiation
    • retarded   (MGI Ref ID J:5968)
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Research Applications
This mouse can be used to support research in many areas including:

Rorasg related

Neurobiology Research
Ataxia (Movement) Defects
Cerebellar Defects
      Purkinje cell defect
Receptor Defects
Tremor Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Rorasg
Allele Name staggerer
Allele Type Spontaneous
Common Name(s) RORalpha-; sg;
Strain of Originobese stock
Gene Symbol and Name Rora, RAR-related orphan receptor alpha
Chromosome 9
Gene Common Name(s) 9530021D13Rik; NR1F1; RIKEN cDNA 9530021D13 gene; ROR1; ROR2; ROR3; RZR-ALPHA; RZRA; Tennessee Mouse Genome Consortium 26; neuroscience mutagenesis facility, 267; nmf267; sg; staggerer; tmgc26;
Molecular Note This allele contains a 6.5kb genomic deletion of an exon encoding part of the ligand binding domain. The deletion results in an exon-skipping event that introduces a shift in the reading frame. The resulting protein is predicted to be truncated due to introduction of a premature stop codon. [MGI Ref ID J:31470]

Genotyping

Genotyping Information

Genotyping Protocols

Rorasg, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Mamontova A; Seguret-Mace S; Esposito B; Chaniale C; Bouly M; Delhaye-Bouchaud N; Luc G; Staels B; Duverger N; Mariani J; Tedgui A. 1998. Severe atherosclerosis and hypoalphalipoproteinemia in the staggerer mouse, a mutant of the nuclear receptor RORalpha. Circulation 98(24):2738-43. [PubMed: 9851961]  [MGI Ref ID J:52105]

SIDMAN RL; LANE PW; DICKIE MM. 1962. Staggerer, a new mutation in the mouse affecting the cerebellum. Science 137:610-2. [PubMed: 13912552]  [MGI Ref ID J:13140]

Additional References

Hamilton BA; Frankel WN; Kerrebrock AW; Hawkins TL; FitzHugh W; Kusumi K; Russell LB; Mueller KL; van Berkel V; Birren BW; Kruglyak L; Lander ES. 1996. Disruption of the nuclear hormone receptor RORalpha in staggerer mice. Nature 379(6567):736-9. [PubMed: 8602221]  [MGI Ref ID J:31470]

Heuze P; Feron C; Baudoin C. 1997. Early behavioral development of mice is affected by staggerer mutation as soon as postnatal day three. Brain Res Dev Brain Res 101(1-2):81-4. [PubMed: 9263582]  [MGI Ref ID J:42144]

Kopmels B; Wollman EE; Guastavino JM; Delhaye-Bouchaud N; Fradelizi D; Mariani J. 1990. Interleukin-1 hyperproduction by in vitro activated peripheral macrophages from cerebellar mutant mice. J Neurochem 55(6):1980-5. [PubMed: 2230805]  [MGI Ref ID J:28095]

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]

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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]

Qiu CH; Shimokawa N; Iwasaki T; Parhar IS; Koibuchi N. 2007. Alteration of cerebellar neurotropin messenger ribonucleic acids and the lack of thyroid hormone receptor augmentation by staggerer-type retinoic acid receptor-related orphan receptor-alpha mutation. Endocrinology 148(4):1745-53. [PubMed: 17218417]  [MGI Ref ID J:129580]

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Saito T; Hirano M; Ide T; Ichiki T; Koibuchi N; Sunagawa K; Hirano K. 2013. Pivotal role of Rho-associated kinase 2 in generating the intrinsic circadian rhythm of vascular contractility. Circulation 127(1):104-14. [PubMed: 23172836]  [MGI Ref ID J:209475]

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Serinagaoglu Y; Zhang R; Zhang Y; Zhang L; Hartt G; Young AP; Oberdick J. 2007. A promoter element with enhancer properties, and the orphan nuclear receptor RORalpha, are required for Purkinje cell-specific expression of a Gi/o modulator. Mol Cell Neurosci 34(3):324-42. [PubMed: 17215137]  [MGI Ref ID J:123239]

Serra HG; Duvick L; Zu T; Carlson K; Stevens S; Jorgensen N; Lysholm A; Burright E; Zoghbi HY; Clark HB; Andresen JM; Orr HT. 2006. RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice. Cell 127(4):697-708. [PubMed: 17110330]  [MGI Ref ID J:116123]

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Soha JM; Herrup K. 1995. Stunted morphologies of cerebellar Purkinje cells in lurcher and staggerer mice are cell-intrinsic effects of the mutant genes. J Comp Neurol 357(1):65-75. [PubMed: 7673468]  [MGI Ref ID J:26094]

Soha JM; Kim S; Crandall JE; Vogel MW. 1997. Rapid growth of parallel fibers in the cerebella of normal and staggerer mutant mice. J Comp Neurol 389(4):642-54. [PubMed: 9421144]  [MGI Ref ID J:44432]

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Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           FGB27

Colony Maintenance

Breeding & HusbandryRorasg/Rorasgmice can be identified by 10-15 days of age by their poor locomotor coordination, lurching movements and frequent falls; homozygotes as young as three days can be distinguished by behavioral testing (Heuze et al.1997). Homozygotes die between two to three weeks of age. Due to their early and high mortality, homozygous mutant mice are not available from our colony. In some cases, the homozygote lifespan can be extended by adding crushed grain to the bottom of the cage and keeping pups with a lactating female. As heterozygotes are indistinguishable phenotypically from wild type mice, they must be identified by a PCR based genotyping assay.
Mating SystemHeterozygote x +/+ sibling         (Female x Male)   13-OCT-11
+/+ sibling x Heterozygote         (Female x Male)   13-OCT-11
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $239.00Female or MaleHeterozygous for Rorasg  
Price per Pair (US dollars $)Pair Genotype
$478.00Heterozygous for Rorasg x Heterozygous for Rorasg  
$311.00Heterozygous for Rorasg x Wild-type for Rorasg  
$311.00Wild-type for Rorasg x Heterozygous for Rorasg  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $310.70Female or MaleHeterozygous for Rorasg  
Price per Pair (US dollars $)Pair Genotype
$621.40Heterozygous for Rorasg x Heterozygous for Rorasg  
$404.30Heterozygous for Rorasg x Wild-type for Rorasg  
$404.30Wild-type for Rorasg x Heterozygous for Rorasg  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   See control note: Wild type mice from the colony are the preferred controls. Untyped mice from the colony may be used; however, heterozygotes exhibit "accelerated loss of Purkinje cells, granule cells, and inferior olivary neurons with age" (MLC; Zanjani et al.1992; Hadj-Sahraoui et al.1997).
   Wild-type from the colony
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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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.

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