Search Criteria: Research Area is "Neurobiology Research: Cerebellar Defects"
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 004146 | B6.129-Tg(Pcp2-cre)2Mpin/J | Repository- Live |
| These transgenic mice express a cre gene inserted into exon 4 of a Pcp2 gene. Mice homozygous for the insert are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Recombinase activity is observed in most Purkinje cells and some retinal bipolar neurons. Small amounts of activity are observed in an unidentified population of cells of the central nervous system tissue. Recombination is first observed around postnatal day 6 and is fully established 2 to 3 weeks after birth. | ||
| 000537 | B6.BR-Agtpbp1pcd/J | Repository- Live |
| Mice homozygous for the Purkinje cell degeneration spontaneous mutation (Agtpbp1pcd) show a moderate ataxia beginning at 3 to 4 weeks. Homozygous mutant mice are somewhat smaller than normal but may live a fairly normal life span. Males have abnormal sperm and are sterile. Females are fertile but are poor breeders. There is rapid degeneration of nearly all Purkinje cells beginning at 15 to 18 days, and a slower degeneration of the photoreceptor cells of the retina and mitral cells of the olfactory bulb. Degeneration of Purkinje cells is followed by partial loss of granule cells. Discrete serotonin-immunoreactive fibers, which ascend to all three layers of the cerebellar cortex in normal controls, are of much higher density and form multidirectional contours in homozygotes. In the retina of homozygous mutant mice, pycnotic nuclei begin to appear in the photoreceptor cells between 18 and 25 days, and the outer rod segments become disorganized. Degeneration of the photor
..... For more information please see the full descriiption on the strain data sheet | ||
| 002651 | B6.C3(Cg)-Rorasg/J | Repository- Live |
| 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 susceptibili
..... For more information please see the full descriiption on the strain data sheet | ||
| 005034 | B6;129-Hcn1tm2Kndl/J | Repository- Live |
| Mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No mRNA encoding the pore/S6 transmembrane domain is detected by in situ hybridization. No HCN1 protein is detected by Western blot analysis of membrane extracts from cerebellum, hippocampus or cortex. Mutant mice exhibit impaired learning capacity in visible platform swimming water maze task and rotorod test, and abnormal eye blink conditioning response. Purkinje cell electrophysiology is abnormal. This mutant mouse strain may be useful in studies of learning, memory and neurophysiology. | ||
| 004200 | B6;CBACa Aw-J/A-Npr2cn-2J/J | Repository- Live |
| Mice carrying the Npr2cn-2J display mutation short thick femurs, round heads, disorganized growth plates, and relatively normal vertebrae; A one month old female exhibited the same phenotype as above but also had splayed ribs. | ||
| 001756 | B6C3Fe a/a-Cacng2stg/J | Repository- Live |
| Mice homozygous for the spontaneous mutation stargazer (Cacng2stg) are first recognizable at 14 days of age by their smaller body size and slightly ataxic gait. Female stargazer homozygous mutant mice are fertile, but most of the males are unable to breed. The mutation is named for the abnormal head movements resembling choreiform head tossing that is characteristic of these mice. The behavioral symptoms progressively worsen with age, but both males and females will live to over one year of age. Electrocorticographical recordings of stargazer mice reveal frequent, prolonged, generalized spike-wave cortical discharges with behavioral arrest. The abnormal brain wave patterns are similar to those seen in human beings with absence epilepsy. Waggler homozygotes (Cacng2stg-wag) manifest a less severe phenotype than stargazer homozygotes, but do display an ataxic gait and occasional seizures. In general, young stargazer mice are more healthy than waggler ho
..... For more information please see the full descriiption on the strain data sheet | ||
| 000636 | B6C3Fe a/a-Lmx1adr-J/J | Repository- Live |
| Lmx1a encodes a LIM homeodomain (LIM-hd) protein that is expressed in the roof plate along the neuraxis during CNS development. The Lmx1adr-J mutation replaces a conserved cysteine in the LIM1 domain of the protein with a tyrosine (Millonig et al. 2000). Mice homozygous for Lmx1adr-Jare identifiable at birth by a thin filament extending from the end of the tail, which drops off within a few days, and a short or blunt tail. They may also have a head bleb. When fur develops, there may be a white belly spot. Lmx1adr-J/Lmx1adr-J mice are ataxic and hyperactive and have difficulty righting themselves, and they do not reproduce (Sweet and Wahlsten 1983). They also exhibit circling behavior, are deaf, and have inner ear and vertebral malformations (Manzanares et al. 2000).
Lmx1adr-J homozygotes exhibit defects of three classes of anatomic structures: the hindbrain roof plate, neural crest derived
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| 000235 | B6C3Fe a/a-Relnrl/J | Repository- Live |
| Mice homozygous for the reeler (Relnrl) mutation exhibit an ataxic gait, dystonic posture and tremors starting around 2 weeks of age. These mutants are incapable of maintaining their hindquarters upright and often fall over during locomotor activity. Moreover, viability and fertility are greatly reduced, especially when the gene is carried on an inbred genetic background. Heterozygotes are visually indistinguishable from wildtype controls. Neuropathies characteristic of Relnrl/Relnrl mutants include a failure of neuronal layer formation in laminated brain regions during development. Neuronal positioning is abnormal within cerebellar, cerebral and hippocampal cortices. The behavioral phenotype is primarily attributed to the severe hypoplasia of the cerebellum, which lacks foliation. Here, there are reduced numbers of granule and Purkinje cells and these cells are aberrantly dispersed among the layers. In the Reln-deficient neo
..... For more information please see the full descriiption on the strain data sheet | ||
| 000501 | B6CBACa Aw-J/A-Aifm1Hq/J | Repository- Live |
| Harlequin mice exhibit paucity of fur resulting in near baldness in hemizygous males and homozygous females. Heterozygous females have a patchy absence of hair that is not always obvious, since the degree of hair loss is notably less than 50%. Homozygotes and hemizygous males weigh less than heterozygous or wild type controls. Ataxia is noticeable by 5 months and progresses as the mice age. Initially the ataxia manifests itself as a side-to-side, unsteady gait with a lateral tremor visible at rest. A delayed cerebellar cortical atrophy has been characterized in these mutants, with an apoptotic loss of granule cells beginning at 4 months of age and a necrotic loss of Purkinje cells occurring subsequently. The granule cells re-enter the cell cycle, but the Purkinje cells do not, supporting the postulate that inappropriate cell cycle re-entry of terminally differentiated neurons can induce apoptosis. Cell loss is greater in the caudal lobules of the cerebellum and is extensive by 9 to 11
..... For more information please see the full descriiption on the strain data sheet | ||
| 003237 | BALB/cByJ-Agtpbp1pcd-3J/J | Repository- Live |
| Mice homozygous for the Purkinje cell degeneration 3 Jackson spontaneous mutation (pcd3J) have an external and histological phenotype that is very similar to the original Purkinje cell degeneration mutation (pcd). Homozygous pcd mutant mice show a moderate ataxia beginning at 3 to 4 weeks, are somewhat smaller than normal but may live a fairly normal life span. Males have abnormal sperm and are sterile. Females are fertile but are poor breeders. There is rapid degeneration of nearly all Purkinje cells beginning at 15 to 18 days, and a slower degeneration of the photoreceptor cells of the retina and mitral cells of the olfactory bulb. Degeneration of Purkinje cells is followed by partial loss of granule cells. Discrete serotonin-immunoreactive fibers, which ascend to all three layers of the cerebellar cortex in normal controls, are of much higher density and form multidirectional contours in homozygotes. In the retina of homozygous mutant mice, pycnotic
..... For more information please see the full descriiption on the strain data sheet | ||
| 008292 | BALB/cJ-Agtpbp1pcd-8J/J | Repository- Live |
| 003092 | BALB/cNctr-Npc1m1N/J | Repository- Live |
| Mice homozygous for the recessive NIH allele of the Niemann Pick type C1 gene (Npc1m1N) show a dual deficiency of sphingomyelinase and glucocerebrosidase activity. The overall phenotype resembles the sphingomyelinosis condition seen in mice homozygous for the sphingomyelinosis allele (Npc1spm, Stock No. 002760). Sphingomyelinosis mutant mice begin to lose weight and to show tremor and ataxic gait at about 7 weeks of age. Weight loss continues and tremor and ataxia become more severe until death at about 12 to 14 weeks of age. The liver and spleen are also enlarged and Purkinje cells in the cerebellum are severely depleted. Sphingomyelin and free cholesterol are markedly elevated in liver and spleen but not in brain. Sphingomyelinosis closely resembles that of human Niemann-Pick Type C disease patients. | ||
| 004683 | C3H/HeSnJ-Slc12a6gaxp/J | Repository- Live |
| Giant axonopathy (gaxp) is an autosomal recessive mutation that arose spontaneously in the Mouse Mutant Resource colony at The Jackson Laboratory. It occurred in a strain bearing another mutation on the C3H/HeDiSnJ background. Homozygous mutants exhibit ataxia of the hind legs with a slight side - to - side wobble. Histopathologic studies showed pale staining, vacuolated structures measuring between 20 and 150 micra in deep cerebellar nuclei, pons, lateral vestibular nuclei and dorsal root and trigeminal ganglia. Ultrastructurally these structures were often bounded by a few layers of myelin, suggesting that they are swollen axons. gaxp has been mapped to Chromosome 2. The most likely gene order places the mutation between D2Mit128 and D2Mit102 in 174 meioses tested. | ||
| 007892 | C57BL/6J-Relnrl-7J/J | Repository- Live |
| 003925 | MRL.129P2(B6)-B2mtm1Unc/Dcr-Dab1scm-2J/J | Repository- Live |
| See Stock No. 000486 for important information on the MRL/MpJ background. | ||
| 002043 | STOCK A/A-Dab1scm/J | Repository- Live |
| Mice homozygous for the scrambler spontaneous mutation (Dab1scm) are recognized by an unstable gait and whole-body tremor. The cerebella of 30-day-old scrambler homozygotes are hypoplastic and devoid of folia; however, neither seizures nor abnormal brain wave patterns have been observed. Scrambler is similar to the reeler mutation in phenotype and pathology and, like reeler, probably results from defective neuronal migration. Female homozygotes mate and breed. Homozygous scrambler mutants have an ataxic gait which in the male may be contributory factor in the failure to mate. Normal life span for both sexes. | ||
| 007684 | STOCK Tg(Atoh1-cre/ESR1)14Fsh/J | Repository- Live |
| Mice hemizygous for this Math1-CreERT2 transgene are viable and fertile. Under control of the Math1 (Atoh1) enhancer, tamoxifen-inducible cre activity is observed in neural progenitors of the cerebellar rhombic lip, dorsal hindbrain and spinal cord, as well as in inner-ear primordia (with a limited amount of ectopic expression in the primordium of the hippocampus but not the cortex). The Cre-ERT2 fusion protein consists of Cre recombinase fused to a triple mutant form of the human estrogen receptor; which does not bind its natural ligand (17b-estradiol) at physiological concentrations but will bind the synthetic estrogen receptor ligands 4-hydroxytamoxifen and, with lesser sensitivity, ICI 182780. Restricted to the cytoplasm, Cre-ERT2 can only gain access to the nuclear compartment after exposure to tamoxifen. To counteract the mixed estrogen agonist effects of tamoxifen injections, which can result in late fetal abortions in pregnant
..... For more information please see the full descriiption on the strain data sheet | ||
| 006207 | STOCK Tg(Pcp2-cre)1Amc/J | Repository- Live |
| Hemizygous mice are viable, fertile, normal in size, and do not display any gross physical or behavioral abnormalities. Cre activity is observed in parasagittal domains of the cerebellum beginning at embryonic day 17 (E17). At E19, low level expression is observed in the most rostral lobe of cerebellum and expression broadens until all Purkinje cells express the transgene in adult mice. When bred with any mouse containing a loxP-flanked sequence of interest, Cre-mediated recombination will result in deletion of the flanked genome. These transgenic mice may be useful in studies utilizing “Cre-lox” technology, specifically regarding the nervous system, development and patterning of cerebellum, and cerebellar hypotrophy converse of Lhermitte-Duclos. | ||
| 003486 | 129-Cstbtm1Rm/J | Repository-Cryopreserved |
| The progressive myoclonus epilepsies (PMEs) types are diseases characterized by tonic-clonic seizures, myoclonic seizures and progressive neurological dysfunction, including dementia and ataxia. Mice lacking cystatin B develop myoclonic seizures and ataxia, similar to symptoms seen in one of these human diseases. Specifically, this mouse is a model of Unverricht-Lundborg disease (EPM1).Strain background has an effect on phenotype. | ||
| 003117 | 129S-Ssttm1Ute/J | Repository-Cryopreserved |
| Mutant mice are healthy, fertile and of normal body size despite having three times the plasma growth hormone levels than wildtype littermates. Somatostatin is virtually absent in the adult cerebellum but is expressed during development. There is impaired motor learning and motor coordination as demonstrated in accelerating rotorod testing. | ||
| 000593 | B6 x B6CBCa Aw-J/A-Grid2Lc T(2;6)7Ca MitfMi-wh/J | Repository-Cryopreserved |
| Mice homozygous for the MitfMi spontaneous mutation are characterized by decreased macrophage chemotactic responses, impaired proliferative responses to B cell and T cell mitogens, diminished responses in vitro to T-dependent and T-independent antigens and reduced NK cell activity. | ||
| 002440 | B6 x BALB/cByJ-Grid2Lc-J/J | Repository-Cryopreserved |
| 003079 | B6.129-Calb1tm1Mpin/J | Repository-Cryopreserved |
| Mice homozygous for the Calb1tm1Mpin targeted mutation are viable and fertile. Homozygous mutant mice show severe impairment in tests of motor coordination and exhibit ataxia when challenged on a runway. These results suggest functional deficits in cerebellar pathways. Confocal calcium imaging of cerebellar Purkinje cells reveals marked changes of synaptically-evoked postsynaptic calcium transients. | ||
| 004068 | B6.129-Iduatm1Clk/J | Repository-Cryopreserved |
| At birth, mice that are homozygous null for the Idua gene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No alpha-L-iduronidase enzyme activity or mRNA is detected. By three weeks of age, homozygous null mice develop a flattened facial profile and a thickening of digits. Defective bone formation is noticeable by fifteen weeks, characterized by a broadening and thickening of long bones. Evidence of lysosomal storage disorder is apparent in cells of the reticuloendothelial system at four weeks. By eight weeks progressive evidence of lysosomal storage is seen in Kupffer cells, splenic sinusoidal lining cells, chondrocytes, glial and Purkinje cells. This animal model is suitable for use in studies investigating the lysosomal storage disorder mucopolysaccharidosis type I (MPS I). | ||
| 005601 | B6.129S-Atxn1tm1Hzo/J | Repository-Cryopreserved |
| Mice that are heterozygous for the targeted mutation are viable but have a reduced lifespan (34-40 weeks). The allele consists of a 154 CAG trinucleotide repeat unit placed within exon 8 of the targeted endogenous mouse locus. Modified transcripts and protein can be detected in brain tissue. By 8 weeks of age, mutant mice exhibit noticeable growth retardation. Progressive neurological degeneration initiates by 9 weeks of age, when mutant mice begin to exhibit a clasping phenotype when held by the tail. By 20 weeks of age muscle wasting, ataxia and an abnormal gait are observed. A lack of motor coordination is detected via an accelerating rotarod test by 5 to 7 weeks. Cognitive defects include poor spatial learning performance and reduced Pavlovian conditioned fear response (impaired memory). Hippocampal basal synaptic function is impaired. Immunohistochemical and immunofluorescent analysis of brain tissue reveals neuronal intranuclear inclusions by 6 weeks of age. Older animals exhibit
..... For more information please see the full descriiption on the strain data sheet | ||
| 003596 | B6.129S4-Cdk5tm1Kul/J | Repository-Cryopreserved |
| Cdk5 is an important molecule for brain development and neuronal differentiation. Cdk null mice exhibit unique lesions in the central nervous system associated with perinatal mortality. The brains of mutant mice lack cortical laminar structure and cerebellar foliation. The large neurons in the brain stem and in the spinal cord show chromatolytic changes with accumulation of neurofilament immunoreactivity. They also exhibit neuronal migration abnormalities, cerebellar defoliation, NF accumulation neuronal bodies, and degenerated motor neurons. In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. This is the case for the strain above. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available. | ||
| 004163 | B6.129S4-Cdk5r1tm1Lht/J | Repository-Cryopreserved |
| At birth, mice homozygous for this targeted allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Cdk5r protein product is not immunodetectable in homozygote whole-brain lysates. Histological analysis of homozygotes reveals severe defects in the patterning of the cerebral cortex and other areas of the brain. The normal lamination pattern of cortical neurons is disrupted; axonal trajectories and dendritic structures are altered. Homozygous mice are more susceptible to mortality from chemically induced-seizures. Sporadic adult lethality is also observed, presumably resulting from spontaneous seizures, a consequence of the disrupted cytoarchitecture observed in the cortex. This mutant mouse strain represents a model that may be useful in studies related to the mechanisms of cortical lamination, epilepsy, Alzheimer's and other neurodegenerative diseases. | ||
| 005970 | B6.129S7-Atoh1tm2Hzo/J | Repository-Cryopreserved |
| Mice that are heterozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Homozygous mutant mice have a perinatal lethal phenotype and die shortly after birth. No gene product (protein) is detected in resting chondrocytes by immunohistochemical analysis of embryonic age 18.5 homozygotes. Beta-galactosidase X-gal staining of neural tissue from embryonic day 14.5 and newborn (postnatal day 0) aged homozygous and heterozygous mice mimicks the endogenous expression pattern. Mice homozygous for this mutation exhibit a phenotype similar to the phenotype observed in mice homozygous for the null (loss of function) targeted mutation. Homozygotes lack cerebellar granule neurons, cochlear and ventricular hair cells, and the pontine nuclei in the brain stem. This mutant mouse strain may be useful in studies of brain and inner ear development. | ||
| 004341 | B6.129X-Cxcr4tm1Qma/J | Repository-Cryopreserved |
| Mice that are homozygous null for the Cxcr4 gene die perinatally, with ~30% dying by embryonic day 18.5. Viable embryos are slightly smaller than wild type mice and exhibit vascular congestion in the kidneys, interstitial hemorrhages, and abnormalities in bone marrow and cerebellum. Homozygote embryos also show reduced B-lymphopoiesis, reduced myelopoiesis in fetal liver and an absence of myelopoiesis in bone marrow. Histological examination reveals a distorted architecture in the tissues if the cerebellum, featuring an attenuated external granule cell layer and an ectopic placement of Purkinje cells. Cxcr4 encodes a receptor commonly called CXCR4, the ligand of which is the chemokine stromal cell-derived factor 1 (SDF-1), an important regulator of hematopoietic cell development, migration and proliferation. CXCR4 also functions as a coreceptor for the entry of T-tropic strains of HIV-1 into CD4+ T cells. In an attempt to offer alleles on well-charac
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| 004202 | B6.C3 Pde6brd1 Hps4le/+ +-Lmx1adr-8J/J | Repository-Cryopreserved |
| 001772 | B6.C3-stu/J | Repository-Cryopreserved |
| Mice homozygous for the stumbler spontaneous mutation (stu) have clinical features suggesting a cerebellar defect. They can be recognized at 12 days of age by a stumbling locomotion characterized by a high-stepping broad-based gait. Homozygous mutant mice become less active with age and progressively smaller than their normal sibs and usually die before weaning. There are fewer than normal Purkinje cells and granule cells from about 10 days of age onward and a consequent smaller size of the cerebellum. The Purkinje cells have small dendritic arborizations and immature spines on their somata. They also have an increased number of mitochondrial profiles both in cell bodies and in swellings on dendrites. The morphology of the granule cells appears normal. | ||
| 002560 | B6.Cg-Aarssti/J | Repository-Cryopreserved |
| Mice homozygous for this spontaneous mutation exhibit a rough, sticky coat, progressive ataxia and Purkinje cell degeneration. Cerebellar Purkinje cell loss is first observed by three weeks of age becomes extensive by six weeks of age and continues to progress slowly over the course of a year. This mutant mouse strain may be useful in studies related to neurodegeneration, protein misfolding and aberrant transfer RNAs. | ||
| 000566 | B6.Cg-Os +/+ Cacna1atg-la/J | Repository-Cryopreserved |
| Mice homozygous for the leaner spontaneous mutation (Cacna1atg-la) begin to show ataxia, stiffness, and retarded motor activity by 8 to 10 days of age. Many homozygous mutant mice die by weaning age, but some survive, and females may even breed. Homozygous mutant adults are characterized by instability of the trunk and hypertonia of the trunk and limb muscles. Seizures have not been observed. The cerebellum is reduced in size, particularly in the anterior region. There is loss of granule cells beginning at 10 days of age and loss of Purkinje and Golgi cells beginning after 1 month. Leaner/tottering heterozygotes (Cacna1atg-la/Cacna1atg) show ataxia, stiffness, and retarded motor activity at 15 to 17 days of age. Within a few days, they develop a wobbly gait and intermittent focal seizures which continue throughout life. The cerebellum shows shrinkage and degenerative changes of the Purkinje cells. | ||
| 000285 | B6.Cg-Rorasg + +/+ Myo5ad Bmp5se/J | Repository-Cryopreserved |
| 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 this congenic strain the staggerer mutation is maintain
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| 000518 | B6.Cg-Usp14ax-J/J | Repository-Cryopreserved |
| The first outward sign of homozygosity for the recessive mutation Usp14ax-J is an unsteady gait, particularly in the hind quarters, that can be detected by approximately 2 weeks of age. These pups often show difficulty in righting themselves when turned on their backs. They have a rapid tremor that is most apparent when they are active. Extensor paralysis progresses and wasting of the hind quarters ensues until the hind quarters are not functional and the mouse can not sit upright or move except through the use of onlyits front limbs. Although death is premature, viability in the first two to three weeks is not diminished. At 18 days of age these mutants weigh an average of 15% less than wildtype controls and by 45 days this difference is increased such that they weigh 50-60% less than wildtype controls. Homozygotes do not breed. The lumbar vertebrae are slightly shorter than normal with shorter spinous processes and more elongate foramen. These homozygotes have defec
..... For more information please see the full descriiption on the strain data sheet | ||
| 000544 | B6.D2-Cacna1atg/J | Repository-Cryopreserved |
| Mice homozygous for the tottering spontaneous mutation (Cacna1atg) are characterized by a wobbly gait beginning at around 3 to 4 weeks of age and affecting, particularly, the hindquarters and by intermittent, spontaneous seizures. The seizures are of two kinds: i) Sudden arrests of movement (behavioral absence seizures) begin at about 3 weeks and are accompanied by abnormal bursts of bilaterally synchronous spike waves in ECG; they last about 0.3 to 10 seconds, occur hundreds of times per day, and continue throughout life; ii) Stereotyped partial motor seizures begin at about 4 weeks and are accompanied by abnormal ECG activity; they last 20 to 30 minutes, occur once or twice a day, and persist throughout life. Homozygous mutant mice of both sexes are fertile, but breeding performance is poor. Leaner/tottering heterozygotes (Cacna1atg-la/Cacna1atg) show ataxia, stiffness, and retarded motor activity at 15 to 17 days of age. Within a few
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| 003799 | B6.D2-Scn8amed-jo/J | Repository-Cryopreserved |
| 001883 | B6.MRL-Cacng2stg-wag/J | Repository-Cryopreserved |
| 003536 | B6;129-Cdk5tm1Kul/J | Repository-Cryopreserved |
| Cdk5 is an important molecule for brain development and neuronal differentiation. Cdk null mice exhibit unique lesions in the central nervous system associated with perinatal mortality. The brains of mutant mice lack cortical laminar structure and cerebellar foliation. The large neurons in the brain stem and in the spinal cord show chromatolytic changes with accumulation of neurofilament immunoreactivity. They also exhibit neuronal migration abnormalities, cerebellar defoliation, NF accumulation neuronal bodies, and degenerated motor neurons. | ||
| 005048 | B6;129-nmf291/J | Repository-Cryopreserved |
| By approximately 11 weeks of age mice homozygous for this ENU-induced mutation develop splayed hind feet, a lumbering gait, and jump into the air intermittently. The phenotype progresses to include spasms of the hind and front limbs that can result in falling over. Both female and male homozygotes breed, but the reproductive window is shortened by the progressive physical impairments. For additional information on nmf291 view the web page on the Neuroscience Mutagenesis Facility web site. | ||
| 003524 | B6;129S6-Lrp8tm1Her/J | Repository-Cryopreserved |
| Mutant mice have a targeted mutation of the apolipoprotein E receptor Lrp8 (also called Apoer2). Homozygotes are viable with no gross morphological abnormalities. Homozygous males have reduced fertility, while females are not affected. Using C-terminal specific antibodies, no endogenous protein is detected in the brains of homozygotes. Homozygous mice have a smaller and less foliated cerebellum with various hippocampus defects in granule cell positioning, cortical neuron migration, granule cell laminar organization, commissural fiber distribution, CA1 microtubule-associated protein 2 (MAP-2) distribution, and long term potentiation (LTP). Mutant mice show contextual fear conditioning deficits. These mice may be useful in studies of brain development, neuronal cytoarchitecture, Reelin signaling pathways, NMDA receptor activity, lipoprotein receptors, synaptic plasticity and learning, schizophrenia, and neurodegenerative disorders such as Alzheimer's disease. | ||
| 005463 | B6;CByJ-Scn8a7J/J | Repository-Cryopreserved |
| View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for Scn8a7J entry. | ||
| 001037 | B6C3Fe a/a-Agtpbp1pcd/J | Repository-Cryopreserved |
| Mice homozygous for the Purkinje cell degeneration spontaneous mutation (pcd) show a moderate ataxia beginning at 3 to 4 weeks. Homozygous mutant mice are somewhat smaller than normal but may live a fairly normal life span. Males have abnormal sperm and are sterile. Females are fertile but are poor breeders. There is rapid degeneration of nearly all Purkinje cells beginning at 15 to 18 days, and a slower degeneration of the photoreceptor cells of the retina and mitral cells of the olfactory bulb. Degeneration of Purkinje cells is followed by partial loss of granule cells. Discrete serotonin-immunoreactive fibers, which ascend to all three layers of the cerebellar cortex in normal controls, are of much higher density and form multidirectional contours in homozygotes. In the retina of homozygous mutant mice, pycnotic nuclei begin to appear in the photoreceptor cells between 18 and 25 days, and the outer rod segments become disorganized. Degeneration of the photoreceptor cells proc
..... For more information please see the full descriiption on the strain data sheet | ||
| 000246 | B6C3Fe a/a-Pitpnavb/J | Repository-Cryopreserved |
| Mice homozygous for the vibrator spontaneous mutation (Pitpnvb) are recognizable at 10 to 12 days of age by a fine rapid tremor. This is followed by a degenerative phase in which there is progressive development of ascending motor paralysis and coarse cerebellar tremor, and finally a terminal phase in which there is loss of consciousness and death. Death occurs by 30 days of age in vibrator mutant mice on an inbred genetic background but many derived from outcrosses may live to 6 months. Mammary glands in homozygotes exhibit underdeveloped alveolar and ductal structures and the fat pad is composed predominantly of brown adipose tissue (Monaco et al., 2004). Neutral lipids are increased two to four fold in the livers of homozygotes (Monaco et al., 2004). The expression of phosphatidylinositol transfer protein alpha is decreased 65-85% compared to wildtype littermates (Monaco et al., 2004). | ||
| 000237 | B6C3Fe a/a-Rorasg/J | Repository-Cryopreserved |
| 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. | ||
| 001607 | B6C3Fe a/a-Unc5crcm/J | Repository-Cryopreserved |
| The cerebellum of mice homozygous for the rostral cerebellar malformation spontaneous mutation (Unc5crcm) is smaller with fewer folia, there are ectopic cerebellar cells in the midbrain, and abnormal neuronal migration. Homozygous mutant mice are ataxic and experience growth retardation early in life. Homozygous males usually do not breed. | ||
| 000243 | B6C3Fe a/a-Wnt1sw/J | Repository-Cryopreserved |
| Mice homozygous for the swaying spontaneous mutation (Wnt1sw) sway to one side or the other when attempting to move and may then pivot clockwise or counterclockwise around their rear legs. Homozygous mutant mice display marked ataxia and hypertonia that is probably attributable to malformations of the anterior vermis of the cerebellum and of the colliculi. The cerebellum is divided on the midline by a deep dorsal sagittal fissure extending from the leptomeninges down to the level of the fourth ventricle. The probable cause is failure of the midline fusion of the cerebellum. | ||
| 001046 | B6CBACa Aw-J/A-Grid2Lc/J | Repository-Cryopreserved |
| Mice heterozygous for the lurcher spontaneous mutation (GridLc) show a characteristic swaying of the hindquarters and a jerky up and down movement. They are identifiable with sureness by their behavior at 12 to 14 days of age. Homozygous mutant micedie shortly after birth but have no visible abnormalities and show severe postnatal loss of Purkinje cells and granule cells. Virtually no Purkinje cells are found in adults and granule cells are reduced to about 10% of normal. The number of neurons in the inferior olivary nucleus falls to about 25% of normal. Other cell populations are normal. The Lc mutation induces apoptotic programmed death of the cerebellar cortical Purkinje cells. Homozygous mutant mice are reproducibly deficient in defined cell populations and thus have been used to study cerebellar function and the distribution of various brain components on cerebellar cells. | ||
| 000247 | B6CBACa Aw-J/A-Kcnj6wv/J | Repository-Cryopreserved |
| 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
..... For more information please see the full descriiption on the strain data sheet | ||
| 003756 | BALB/cByJ-nr/J | Repository-Cryopreserved |
| On the BALB/cByJ background nervous homozygotes have a delayed expression of the mutant phenotype relative to that on the C3HeB/FeJ background (see Stock No. 000229). At 8 to 9 weeks of age homozygotes have a ruffled and disheveled appearance and are slightly smaller than their affected littermates. They are ataxic, having an unsteady gait particularly in the hindquarters, but do not display tremors. They are more active than their littermates. Males and females are affected equally and have a normal lifespan. These mice are good breeders with few non-productive matings. A cross between C3Fe.CGr(Cg)-nr/J homozygotes and BALB/cByJ-nr/J homozygotes produced compound heterozygotes most of which presented with the nervous phenotype by 4 weeks of age, although some had onset delayed as much as to 7 weeks of age (Giggey and Thompson, 2005). There is a 90% loss of Purkinje cells between 23 and 50 days in homozygous mu
..... | ||
| 001049 | BKS.Cg-mea2J m/+ +/J | Repository-Cryopreserved |
| Mice homozygous for the meander tail 2J spontaneous mutation (mea2J) have a phenotype that is very similar to the original meander tail (mea) and meander tail J mice (meaJ). Homozygous mutant mice have variably shortened and kinked tails. At about 2 weeks an unsteadiness of gait, often amounting to a tremor, appears. Homozygotes are smaller and less vigorous than wiltype controls but are fertile. The anterior lobe of the cerebellum is disorganized. Purkinje cells are scattered, some cellular layers never form, and Bergmann glial processes are absent. The cytoarchitecture of the posterior lobe is normal, and the transition from normal to disorganized areas is sharply demarcated. This strain is maintained in linkage with the misty coat color mutation (m). | ||
| 001192 | BKS.Cg-meaJ Leprdb +/+ + m/J | Repository-Cryopreserved |
| Mice homozygous for the meander tail-J spontaneous mutation (meaJ) have variably shortened and kinked tails. At about 2 weeks an unsteadiness of gait, often amounting to a tremor, appears. Homozygous mutant mice are smaller and less vigorous than wiltype controls but are fertile. The anterior lobe of the cerebellum is disorganized. Purkinje cells are scattered, some cellular layers never form, and Bergmann glial processes are absent. The cytoarchitecture of the posterior lobe is normal, and the transition from normal to disorganized areas is sharply demarcated. This mutation occurred in the BKS.Cg-Leprdb +/+ m strain so it is also segregating for the diabetes (Leprdb) and misty (m) mutations. See strain description for BKS.Cg-Leprdb +/+ m (Stock No. 000642) for more information. | ||
| 000229 | C3Fe.CGr(Cg)-nr/J | Repository-Cryopreserved |
| Nervous homozygotes on this C3HeB/FeJ congenic background display a mutant phenotype by 3 to 4 weeks of age, much earlier than on the BALB/cByJ background (see Stock No. 003756). Because this earlier onset occurs at wean age, the affected pups may have to stay with the mother an extra week or two. Homozygotes are somewhat smaller than their littermates and are ataxic with a slight head bobbing motion but do not display tremors. They often fall over on their side and have a sort of backwards lurching movement. They have a tendency to look up and are hyperactive compared to their littermates. Males and females are equally affected and have a normal lifespan. They are poor breeders with a higher than normal incidence of non-productive matings. A cross between C3Fe.CGr(Cg)-nr/J homozygotes and BALB/cByJ-nr/J homozygotes produced compound heterozygotes most of which presented with the nervous phenotype by 4 weeks of age,
..... For more information please see the full descriiption on the strain data sheet | ||
| 002235 | C3H/HeSnJ-Ctnna2cdf/J | Repository-Cryopreserved |
| Cerebellar deficient folia (cdf) is a recessive mutation which maps to chromosome 6. Homozygotes display a constant side-to-side wobble in their gait that is apparent as early as 2 weeks of age. Additionally, they often hold their tails off the surface or arch them over their heads while moving. Smaller size is detectable in homozygotes by two weeks of age, at weaning they weigh approximately 25% less than littermate controls, and as adults they weigh, on average, 50% less. These mutants live a normal life span (one-and-a-half to two years on the C3H/HeJ background), but homozygous males rarely breed and homozygous females are poor mothers requiring transfer of litters to foster mothers. The cdf mutation causes a hypoplastic and dysmorphic cerebellum. On the C3H/HeJ background, homozygotes have only 7 cerebellar folia rather than the 10 present in wildtype, and the folia pattern is abnormal. Their vermis is one-third smaller than wildtype in rostro-caudal length
..... | ||
| 000527 | C57BL/6J-Grid2ho-5J/J | Repository-Cryopreserved |
| 004817 | C57BL/6J-Npc1nmf164/J | Repository-Cryopreserved |
| View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for Npc1nmf164 entry. | ||
| 005744 | C57BL/6J-Relnrl-6J/J | Repository-Cryopreserved |
| View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for Relnrl-6J entry. | ||
| 005047 | C57BL/6J-Rorasg-3J/J | Repository-Cryopreserved |
| Mice homozygous for this ENU-induced mutation are smaller than normal and, by 3.5 weeks of age on average, display splayed hind limbs and a leaning gait. They fall over on their sides and have difficulty regaining a walking position. A disorganized Purkinje cell layer and loss of granule cells has been found. For additional information on Rorasg-3J view the web page on the Neuroscience Mutagenesis Facility web site. | ||
| 004823 | C57BL/6J-nmf205/J | Repository-Cryopreserved |
| View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for nmf205 entry. | ||
| 002760 | C57BLKS/J-Npc1spm/J | Repository-Cryopreserved |
| The sphingomyelinosis mutation (Npc1spm) arose in the C57BLKS inbred strain. Homozygous mutant mice begin to lose weight and to show tremor and ataxic gait at about 7 weeks of age. Weight loss continues and tremor and ataxia become more severe until death at about 12 to 14 weeks of age. The liver and spleen are also enlarged and Purkinje cells in the cerebellum are severely depleted. Sphingomyelin and free cholesterol are markedly elevated in liver and spleen but not in brain. Sphingomyelinosis closely resembles that of human Niemann-Pick Type C disease patients. A dual deficiency of sphingomyelinase and glucocerebrosidase activity has been described in BALB/c mice (npc1N) with resemblance to the sphingomyelinosis condition. | ||
| 003581 | CBy.129S4-Dab1tm1Cpr/J | Repository-Cryopreserved |
| Homozygous Dab1-null mice become tremulous and ataxic at approximately postnatal day 10. Multiple defects can be detected in brain tissue. Lamellar structures in the cortex and hippocampus appear disorganized and the cerebellum is small and disorganized. Although homozygotes null reportedly die at 4-5 weeks on a mixed B6,129 or 129/Sv background, mice on a BALB/cByJ are viable. | ||
| 004913 | CByJ.B6-Inpp4awbl/FrkJ | Repository-Cryopreserved |
| Mice homozygous for Inpp4awbl exhibit small size, ataxia, and cell death in the cerebellum, hippocampus (CA1) and neocortex. Homozygotes die between two and five weeks probably from seizures and/or failure to thrive. Heterozgotes do not display an overt locomotor defect. | ||
| 004518 | DBA/2J-Agtpbp1pcd-5J/J | Repository-Cryopreserved |
| 000548 | DBA/2J-Grid2ho-4J/J | Repository-Cryopreserved |
| 000253 | DLS/LeJ | Repository-Cryopreserved |
| Mice homozygous for the dilute-lethal spontaneous mutation (Myo5ad-l) display a severe neuromuscular disorder characterized by convulsions and opisthotonus. Homozygous mutant mice usually die by approximately 3 weeks of age. Dilute lethal homozygotes lack smooth endoplasmic reticulum in the dendritic spine of Purkinje cells causing an absence of intracellular calcium. Loss of this intracellular calcium may be the cause of dilute-lethal neurological symptoms. Homozygous dilute-neurological mutant mice (Myo5ad-n, Stock No. 001013) display a neuromuscular disorder, but the condition is less severe than in dilute-lethal homozygotes. In this DLS/Le inbred strain the dilute-lethal mutation is maintained in repulsion with short ear (Bmpse), closely linked mutations on Chromosome 9. | ||
| 005625 | FVB-Tg(Pcp2-tTA)3Horr/J | Repository-Cryopreserved |
| Mice that are homozygous for the transgene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Cerebellar Purkinje cell-specific tTA expression has been confirmed using PCR. Constitutive expression of tTA in Purkinje cells makes this strain suitable for creating bitransgenic mice that can, by withdrawing tetracycline (or its derivative doxycycline), inducibly express a gene of interest in Purkinje cells when the gene of interest is under the direction of a tetracycline-responsive element (TRE; tetO). | ||
| 004083 | NOD.129(B6)-Prkdcscid Iduatm1Clk/J | Repository-Cryopreserved |
| At birth, mice that are homozygous null for the Idua gene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No alpha-L-iduronidase enzyme activity or mRNA is detected. By three weeks of age, homozygous null mice develop a flattened facial profile and a thickening of digits. Defective bone formation is noticeable by fifteen weeks, characterized by a broadening and thickening of long bones. Evidence of lysosomal storage disorder is apparent in cells of the reticuloendothelial system at four weeks. By eight weeks progressive evidence of lysosomal storage is seen in Kupffer cells, splenic sinusoidal lining cells, chondrocytes, glial and Purkinje cells. This animal model is suitable for use in studies investigating the lysosomal storage disorder mucopolysaccharidosis type I (MPS I). | ||
| 002238 | STOCK a Tyrp1b shmy/J | Repository-Cryopreserved |
| Shimmy homozygotes can generally be identified by 12-14 days of age by a hesitant, wobbly gait with swaying hindquarters and slightly smaller overall body size as compared with unaffected littermates. There can be a delay in the opening of the eyes such that one or both remain closed until 15 to 20 days of age. | ||
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