Strain Name: |
B6CBACa Aw-J/A-Grid2Lc/J |
|---|---|
Stock Number: |
001046 |
Availability: | Repository-Cryopreserved |
General Terms and Conditions |
| Former Name |
B6CBACa-Aw-J/A-Grid2Lc (Changed: 15-DEC-04
) |
| Genes & Alleles | Aw-J; Grid2; Grid2Lc; a; |
Product Information
Strain Details
Type JAX® GEMM® Strain - Mutant Strain Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Spontaneous Mutation Species laboratory mouse Generation N60p Appearance
white-bellied agouti, ataxic
Related Genotype: Aw-J/? Grid2Lc/+
agouti, ataxic
Related Genotype: A/A Grid2Lc/+
white-bellied agouti, unaffected
Related Genotype: Aw-J/? +/+
agouti, unaffected
Related Genotype: A/A +/+Strain Description
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.
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Aw-J | ||
|---|---|---|---|
| Allele Name | white bellied agouti Jackson | ||
| Common Name(s) | AWJ; | ||
| Strain of Origin | C57BL/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 | Grid2Lc | ||
| Allele Name | lurcher | ||
| Common Name(s) | Grid2-Lc; lc; | ||
| Strain of Origin | STOCK Mitf | ||
| Gene Symbol and Name | Grid2, glutamate receptor, ionotropic, delta 2 | ||
| Chromosome | 6 | ||
| Gene Common Name(s) | B230104L07Rik; GluRdelta2; Lc; LcJ; MGC117022; MGC117023; MGC117024; RIKEN cDNA B230104L07 gene; cpr; creeper; ho; hotfoot; lurcher; lurcher Jackson; neuroscience mutagenesis facility, 408; nmf408; tapdancer; tpr; | ||
| General Note | Lurcher arose as a spontaneous mutation in a male homozygous for the microphthalmia allele white (Mitfmi-wh). Heterozygotes show a characteristic swaying of the hindquarters and a jerky up and down movement. They are identifiable with certainty by their behavior at 12 to 14 days of age. They are smaller than normal at maturity, but are fertile and have a normal life span (J:289).Homozygotes die shortly after birth. They have no visible abnormalities (J:289), but show severe postnatal loss ofPurkinje 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 (J:6232, J:5786).Wetts and Herrup (J:6841, J:6931), using chimeras of Grid2Lc/+ with wild-type mice in which the lurcher and wild type cells are distinguishable by cytoplasmic or nuclear markers, show that the action of lurcher is intrinsic to Purkinje cells and that the loss of granule cells and olive cells is probably secondary. The same authors conclude, from the numbers of wild type Purkinje cells found in different lurcher--wild type chimeras, that the number of Purkinje cell progenitorsis very small, perhaps 8 to 11 in each brain half, and that each progenitor gives rise to about 10,000 Purkinje cells (J:6866, J:12731). The loss of Purkinje cells in lurcher mutants is intrinsic to the cells, as are the morphological abnormalities of these cells prior to their death (J:26094). The Grid2Lc mutation induces apoptotic programmed death of the cerebellar cortical Purkinje cells (J:24329). Purkinje cells from lurcher animals can, however, survive in vitro (J:26917), and deep cerebellar Purkinje cells survive in vivo (J:25957).Grafting of cerebellar tissues from normal mice into adult Grid2Lc/+ mice, either as solid tissues (J:16367) or as a cell suspension (J:14032), causes a partial, but only a partial, reconstructionof cerebellar neural circuitry. Grafted Purkinje cells invade the dorsal cochlear nuclei of lurcher mice, emphasizing the resemblance of this nucleus to cerebellar tissue in some respects (J:15257).Cerebellar Purkinje cells are the target of climbing fibers originating from granule cells and from inferior olivary neurons. The loss of the cerebellar targets results in a decrease in the afferent neuron populations (J:12661). Initial multiple innervation of Purkinje cells, each receiving several climbing fibers, is transient and is succeeded by a one-to-one relationship in normal cerebellum. Due to an intrinsic incompetence of Grid2Lc/+ Purkinje cells, they retain multiple innervation (J:3428).Lurcher mice have motor abnormalities, but not inall motor function tests (J:990), and motor learning is not slowed (J:17180). Although these mice also show deficits in spatial orientation, the two abnormalities are not correlated (J:22285). There is a massive loss of neurons in the cerebellar cortex, but deep cerebellar nuclei are intact, and are sufficient for motor learning of simple tasks (J:25957).Lurcher mice, reproducibly deficient in defined cell populations, have been used to study cerebellar function and the distribution of various brain components on cerebellar cells. Lurcher has been suggested as a model for a human dominant hereditary ataxia, olivopontocerebellar atrophy (OPCA). Inositol triphosphate metabolism is slowed in Grid2Lc/+ mice and in OPCA patients. This metabolic alteration may be associated with the beginnings of degeneration of the Purkinje cells (J:1412). Insulin-like growth factor 1 binding and receptor phosphorylation is also reduced in both lurcher mice and OPCA patients (J:18719).The lurcher mutation maps to Chr 6 near Mitfmi-wh (J:289). Intersubspecific backcross mapping suggests a somewhat more proximal position (J:11037). A high resolution genetic map and a YAC contig of the lurcher locus contributed significantly to the identification of lurcher as a mutation in Grid2 (J:30546). | ||
| Molecular Note | The lurcher (Grid2Lc) and lurcher-J (Grid2Lc-J) mutations are nucleotide substitutions that cause a change in an amino acid in the third transmembrane domain of Grid2. [MGI Ref ID J:42431] | ||
Control Information
| Allele | Control | |
|---|---|---|
| Grid2Lc | Wild-type from the colony | |
| Considerations for Choosing Controls | ||
Genotyping Protocols
Aw-J
Related Strains
Strains carrying Aw-J allele
View Strains carrying Aw-J (31 strains)
000593 B6 x B6CBCa Aw-J/A-Grid2Lc T(2;6)7Ca MitfMi-wh/J
002440 B6 x BALB/cByJ-Grid2Lc-J/J 005447 C57BL/6J-Grid2ho-16J/J 005718 C57BL/6J-Grid2ho-17J/J 000527 C57BL/6J-Grid2ho-5J/J 000548 DBA/2J-Grid2ho-4J/J
Research Applications
This mouse can be used to support research in many areas including:Grid2Lc related
Developmental Biology Research
Embryonic Lethality (Homozygous)
Neurobiology Research
Ataxia (Movement) Defects
Cerebellar Defects (Purkinje cell defect)
Receptor Defects (glutamate receptor: ionotropic)
References
Additional ReferencesPrice and Supply Information
| Strain Name: | B6CBACa Aw-J/A-Grid2Lc/J |
| Stock Number: | 001046 |
Price Details
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Supply Details
| Standard Supply | Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
Cryorecovery - Standard. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery. Cryorecovery to establish a Dedicated Supply for greater quantities of mice. |
| Licensing | See General Terms and Conditions below |
| Control Information | View Control Information in Strain Details. |
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