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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Strain Information
Former Names B6C3Fe a/a-Unc5h3rcm/J (Changed: 15-DEC-04 ) B6C3Fe-a/a-Unc5h3rcm (Changed: 15-DEC-04 ) Type Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation N3 Description
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.Development
The rostral cerebellar malformation mutation (rcm) arose spontaneously in the B6.C3- Pde6brd1 Hps4le/J stock recovered from cryopreservation at The Jackson Laboratory in 1989 at N40pF1. The stock was sibling mated for 2 generations then maintained by ovarian transplant-cross-intercross. The first cross used ovaries from a homozygous rcm female which were transplanted into a host that was then bred to a C57BL/6J male. The heterozygous offspring were intercrossed and an ovarian transplant was done from a homozygous female, then the host was bred with a C3HeB/FeJ-a/a male and their offspring intercrossed. Ovarian transplant was done from a resulting homozygyous female and the host was bred to B6C3Fe-a/a F1 which became the standard mate in the cross-intercross maintenance of this strain. In 1991 embryos were generated for cryopreservation by breeding N2 heterozygotes.
Control Information
| Control | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying a allele
View Strains carrying a (104 strains)
Phenotype
Phenotype Information
assigned by genotype
Unc5crcm/Unc5crcm
B6C3Fe a/a-Unc5crcm/J
- reproductive system phenotype
- *normal* reproductive system phenotype (MGI Ref ID J:1827)
- males on this hybrid background can sire litters unlike males on an inbred C57BL/6J background
- nervous system phenotype
- abnormal cerebellar Purkinje cell layer (MGI Ref ID J:40243)
- abnormal groups of migrating granule cells are associated with gaps in the Purkinje cell layer
- abnormal Purkinje cell morphology (MGI Ref ID J:45577)
- at E13.5 Purkinje cell progenitors extend slightly more rostrally towards the pontine area
- ectopic Purkinje cell (MGI Ref ID J:40243)
- in the inferior colliculus and tegmentum of the midbrain
- ectopic cells are detected at P3 but not at P0
- at E17.5 Purkinje cells invade the lateral regions of the pontine area
- in the inferior colliculus and pontine area at P7
- scattered randomly through the granule cell layer and cells are also seen in some areas of the molecular layer, all in the parafloccular lobe lateral to the midbrain
- abnormal cerebellar granule layer (MGI Ref ID J:1827)
- variable thickness and cell density in the parafloccular lobe lateral to the midbrain
- throughout the cerebellum small tongue-like extensions of the molecular layer into the granule cell layer are seen
- ectopic granule cells (MGI Ref ID J:40243)
- an abnormal band of granule cells extends from the cerebellum into the inferior colliculus
- ectopic cells are first detected at P3 and increase in number by P7
- in the inferior colliculus and pontine area at P7
- in some areas of the molecular layer in the parafloccular lobe lateral to the midbrain
- abnormal cerebellar molecular layer (MGI Ref ID J:1827)
- throughout the cerebellum small tongue-like extensions of the molecular layer into the granule cell layer are seen
- abnormal cerebellum development (MGI Ref ID J:40243)
- an abnormal band of granule cells extends from the cerebellum into the inferior colliculus
- partial fusion of the inferior colliculus to the rostral cerebellum
- expression analysis identifies neuroprogenitors ectopically located beneath the pia mater and at E17.5 these cells extend more rostrally than the Purkinje cell progenitors
- ectopic cerebellar cells are seen in the inferior colliculus and pontine area at P7
- abnormal cerebellar lobule formation (MGI Ref ID J:40243)
- only 6 lobes are formed
- vermal fissure formation is also abnormal in the lateral cerebral hemisphere
- abnormal external granule cell layer morphology (MGI Ref ID J:40243)
- abnormal external granule cell migration with cells migrating in cohorts rather than as single cells
- these abnormal groups of cells are often associated with gaps in the Purkinje cell layer
- reduced cerebellar foliation (MGI Ref ID J:40243)
- a reduction in size and number of folia is seen in midline sagital sections
- abnormal inferior colliculus (MGI Ref ID J:40243)
- an abnormal band of granule cells extends from the cerebellum into the inferior colliculus
- partial fusion of the inferior colliculus to the rostral cerebellum with ectopic granule and Purkinje cells in the inferior colliculuc and tegmentum of the midbrain
- abnormal neuronal migration (MGI Ref ID J:45577)
- abnormal rostral migration of Purkinje and granule cells into the inferior colliculus
- small cerebellum (MGI Ref ID J:40243)
- less pronounced than in Unc5crcmTg(Ucp)1.23Kz homozygotes
- seen at E17.5
- behavior/neurological phenotype
- abnormal gait (MGI Ref ID J:1827)
- swaying gait seen at 12 days of age
- ataxia (MGI Ref ID J:1827)
- growth/size phenotype
- postnatal growth retardation (MGI Ref ID J:1827)
- smaller than littermates at 12 days of age but catch up in size by 5 - 6 weeks of age
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Unc5crcm/Unc5crcm
C57BL/6J-Unc5crcm
- lethality-postnatal
- postnatal lethality (MGI Ref ID J:1827)
- fewer than expected homozygotes are found at weaning; however, those that survive to weaning have a normal life span
- reproductive system phenotype
- male infertility (MGI Ref ID J:14268)
- males on an inbred C57BL/6J background fail to sire litters however, males on a hybrid B6C3Fe a/a background may sire litters
- nervous system phenotype
- abnormal cerebellum morphology (MGI Ref ID J:46670)
- in lateral sections the cerebellum extends further anteriorly especially in the lateral vermal and paravermal regions
- ectopic cerebellar tissue adheres to the posterior and lateral aspects of the inferior colliculus and along the lateral aspect of the brainstem
- ectopic tissue is present paramedially but not at the midline and does not contain cerebellar nuclear cells
- increased dispersal of cerebellar mossy fiber terminals and additional spinocerebellar mossy fiber terminal bands lateral to the usual 5 bands are seen in the anterior and ectopic cerebellar tissue; however mossy fibers in the posterior region appear similar to wild-type
- abnormal cerebellar cortex morphology (MGI Ref ID J:14268)
- irregular thickness of the anterior lobe
- abnormalities of the layers are more severe in the ectopic tissue
- abnormal cerebellar Purkinje cell layer (MGI Ref ID J:14268)
- Purkinje and granule cells are scrambled
- increasing degree of multilamination as the normal anterior boundary of the cerebellum is approached
- ectopic Purkinje cell (MGI Ref ID J:46670)
- within the granule cell layer in the ectopic cerebellar tissue
- abnormal cerebellar granule layer (MGI Ref ID J:14268)
- Purkinje and granule cells are scrambled
- acellular areas interrupt this layer
- ectopic granule cells (MGI Ref ID J:46670)
- in the medullary regions especially in the medial area of lobule VI
- clusters of ectopic granule cells in the molecular layer in the ectopic cerebellar tissue
- abnormal cerebellar lobule formation (MGI Ref ID J:46670)
- smaller lobule V and less pronounced hemispheric part of lobule VI
- lobule VI does not appear to extend to the lateral aspect of the hemisphere
- lobule IX is reduced and appears to be joined to lobule VIII in the midline region
- the medial cerebellum usually has only 2 lobules rather than 3 as in wild-type mice
- small cerebellum (MGI Ref ID J:46670)
- increased inferior colliculus size (MGI Ref ID J:46670)
- appears to extend further posteriorly
- behavior/neurological phenotype
- abnormal gait (MGI Ref ID J:1827)
- swaying gait seen at 12 days of age
- abnormal posture (MGI Ref ID J:46670)
- broad stance of the hind limbs
- when walking hind limbs and toes are splayed
- ataxia (MGI Ref ID J:14268)
- tend to fall to the side
- tend to fall to one side, lean to one side when walking, and only rear when support is available
- head bobbing (MGI Ref ID J:46670)
- tend to bob up and down or side to side
- tremors (MGI Ref ID J:46670)
- slight
- growth/size phenotype
- postnatal growth retardation (MGI Ref ID J:1827)
- smaller than littermates at 12 days of age but catch up in size by 5 - 6 weeks of age
- hearing/vestibular/ear phenotype
- head bobbing (MGI Ref ID J:46670)
- tend to bob up and down or side to side
This mouse can be used to support research in many areas including:Unc5crcm related
Neurobiology Research
Ataxia (Movement) Defects
Cerebellar Defects
Receptor Defects
Genes & Alleles
Gene & Allele Information
| Allele Symbol | Unc5crcm | ||
|---|---|---|---|
| Allele Name | rostral cerebellar malformation | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | rcm; rcms; | ||
| Strain of Origin | C57BL/6J-Hps4 | ||
| Gene Symbol and Name | Unc5c, unc-5 homolog C (C. elegans) | ||
| Chromosome | 3 | ||
| Gene Common Name(s) | AI047720; B130051O18Rik; RIKEN cDNA B130051O18 gene; UNC-5 homolog (C. elegans) 3; UNC5H3; Unc5h3; expressed sequence AI047720; rcm; rostral cerebellar malformation; unc5 homolog (C. elegans) 3; | ||
| Molecular Note | The mutation underlying the mutant phenotype is a tandem duplication of an exon encoding amino acids 763-818. The transcript expressed from this allele contains an in-frame insertion that is predicted to result in a protein that contains an additional 55 amino acids in the cytoplasmic region. [MGI Ref ID J:40243] | ||
| Allele Symbol | a | ||
| Allele Name | nonagouti | ||
| Allele Type | Spontaneous | ||
Genotyping
Genotyping Information
This strain will not have a genotyping protocol or one is not currently available.
Helpful Links
Genotyping resources and troubleshooting
References
References
Additional References
Goldowitz D; Hamre KM; Przyborski SA; Ackerman SL. 2000. Granule cells and cerebellar boundaries: analysis of Unc5h3 mutant chimeras. J Neurosci 20(11):4129-37. [PubMed: 10818148] [MGI Ref ID J:62291]
Lane PW; Bronson RT; Spencer CA. 1992. Rostral cerebellar malformation, (rcm): a new recessive mutation on chromosome 3 of the mouse. J Hered 83(4):315-8. [PubMed: 1401878] [MGI Ref ID J:1827]
Przyborski SA; Knowles BB; Ackerman SL. 1998. Embryonic phenotype of Unc5h3 mutant mice suggests chemorepulsion during the formation of the rostral cerebellar boundary. Development 125(1):41-50. [PubMed: 9389662] [MGI Ref ID J:45577]
Schwarting GA; Raitcheva D; Bless EP; Ackerman SL; Tobet S. 2004. Netrin 1-mediated chemoattraction regulates the migratory pathway of LHRH neurons. Eur J Neurosci 19(1):11-20. [PubMed: 14750959] [MGI Ref ID J:89800]
Unc5crcm relatedAckerman SL; Kozak LP; Przyborski SA; Rund LA; Boyer BB; Knowles BB. 1997. The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein. Nature 386(6627):838-42. [PubMed: 9126743] [MGI Ref ID J:40243]
Bernet A; Mazelin L; Coissieux MM; Gadot N; Ackerman SL; Scoazec JY; Mehlen P. 2007. Inactivation of the UNC5C Netrin-1 receptor is associated with tumor progression in colorectal malignancies. Gastroenterology 133(6):1840-8. [PubMed: 17967459] [MGI Ref ID J:131645]
Ding YQ; Kim JY; Xu YS; Rao Y; Chen ZF. 2005. Ventral migration of early-born neurons requires Dcc and is essential for the projections of primary afferents in the spinal cord. Development 132(9):2047-56. [PubMed: 15788454] [MGI Ref ID J:98501]
Eisenman LM; Brothers R. 1998. Rostral cerebellar malformation (rcm/rcm): a murine mutant to study regionalization of the cerebellum. J Comp Neurol 394(1):106-17. [PubMed: 9550145] [MGI Ref ID J:46670]
Lane PW; Bronson R; Spencer C. 1990. Rostral cerebellar malformation. Mouse Genome 86:237. [MGI Ref ID J:14268]
Lane PW; Bronson RT; Spencer CA. 1992. Rostral cerebellar malformation, (rcm): a new recessive mutation on chromosome 3 of the mouse. J Hered 83(4):315-8. [PubMed: 1401878] [MGI Ref ID J:1827]
Przyborski SA; Knowles BB; Ackerman SL. 1998. Embryonic phenotype of Unc5h3 mutant mice suggests chemorepulsion during the formation of the rostral cerebellar boundary. Development 125(1):41-50. [PubMed: 9389662] [MGI Ref ID J:45577]
Schwarting GA; Raitcheva D; Bless EP; Ackerman SL; Tobet S. 2004. Netrin 1-mediated chemoattraction regulates the migratory pathway of LHRH neurons. Eur J Neurosci 19(1):11-20. [PubMed: 14750959] [MGI Ref ID J:89800]
Health & husbandry
Health & Colony Maintenance Information
Currently there no information available for this strain. This may be due to the supply level of this strain.
Purchasing information
Pricing, Supply Level & Notes, Controls, General Terms & Conditions
Pricing
| Pricing for USA, Canada and Mexico shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
| Pricing for International shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
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Supply Details
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
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| Supply Notes |
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Control Information
| Control | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| International - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.Ordering and Purchasing Information
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