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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 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 | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
Strains carrying a allele
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View Related Disease (OMIM) Terms
Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested. Skin/Hair/Eye Pigmentation, Variation In, 9; SHEP9 (ASIP)
View Mammalian Phenotype Terms
Mammalian Phenotype Terms provided by MGI
assigned by genotype
Unc5crcm/Unc5crcm
B6C3Fe a/a-Unc5crcm/J
- reproductive system phenotype
- *normal* reproductive system phenotype
- males on this hybrid background can sire litters unlike males on an inbred C57BL/6J background (MGI Ref ID J:1827)
- nervous system phenotype
- abnormal cerebellar Purkinje cell layer
- abnormal groups of migrating granule cells are associated with gaps in the Purkinje cell layer (MGI Ref ID J:40243)
- abnormal Purkinje cell morphology
- at E13.5 Purkinje cell progenitors extend slightly more rostrally towards the pontine area (MGI Ref ID J:45577)
- ectopic Purkinje cell
- in the inferior colliculus and tegmentum of the midbrain (MGI Ref ID J:40243)
- ectopic cells are detected at P3 but not at P0 (MGI Ref ID J:40243)
- at E17.5 Purkinje cells invade the lateral regions of the pontine area (MGI Ref ID J:45577)
- in the inferior colliculus and pontine area at P7 (MGI Ref ID J:45577)
- 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 (MGI Ref ID J:1827)
- abnormal cerebellar granule layer
- ectopic cerebellar granule cells
- an abnormal band of granule cells extends from the cerebellum into the inferior colliculus (MGI Ref ID J:40243)
- ectopic cells are first detected at P3 and increase in number by P7 (MGI Ref ID J:40243)
- in the inferior colliculus and pontine area at P7 (MGI Ref ID J:45577)
- in some areas of the molecular layer in the parafloccular lobe lateral to the midbrain (MGI Ref ID J:1827)
- abnormal cerebellar molecular layer
- throughout the cerebellum small tongue-like extensions of the molecular layer into the granule cell layer are seen (MGI Ref ID J:1827)
- abnormal cerebellum development
- an abnormal band of granule cells extends from the cerebellum into the inferior colliculus (MGI Ref ID J:40243)
- partial fusion of the inferior colliculus to the rostral cerebellum (MGI Ref ID J:40243)
- expression analysis identifies neuroprogenitors ectopically located beneath the pia mater and at E17.5 these cells extend more rostrally than the Purkinje cell progenitors (MGI Ref ID J:45577)
- ectopic cerebellar cells are seen in the inferior colliculus and pontine area at P7 (MGI Ref ID J:45577)
- abnormal cerebellar lobule formation
- abnormal cerebellum external granule cell layer morphology
- reduced cerebellar foliation
- a reduction in size and number of folia is seen in midline sagital sections (MGI Ref ID J:40243)
- abnormal inferior colliculus morphology
- abnormal neuronal migration
- abnormal rostral migration of Purkinje and granule cells into the inferior colliculus (MGI Ref ID J:45577)
- small cerebellum
- behavior/neurological phenotype
- abnormal gait
- swaying gait seen at 12 days of age (MGI Ref ID J:1827)
- ataxia (MGI Ref ID J:1827)
- growth/size phenotype
- postnatal growth retardation
- smaller than littermates at 12 days of age but catch up in size by 5 - 6 weeks of age (MGI Ref ID J:1827)
- cellular phenotype
- abnormal neuronal migration
- abnormal rostral migration of Purkinje and granule cells into the inferior colliculus (MGI Ref ID J:45577)
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Unc5crcm/Unc5crcm
C57BL/6J-Unc5crcm
- mortality/aging
- partial preweaning lethality
- fewer than expected homozygotes are found at weaning; however, those that survive to weaning have a normal life span (MGI Ref ID J:1827)
- 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 (MGI Ref ID J:1827)
- nervous system phenotype
- abnormal cerebellum morphology
- in lateral sections the cerebellum extends further anteriorly especially in the lateral vermal and paravermal regions (MGI Ref ID J:46670)
- ectopic cerebellar tissue adheres to the posterior and lateral aspects of the inferior colliculus and along the lateral aspect of the brainstem (MGI Ref ID J:46670)
- ectopic tissue is present paramedially but not at the midline and does not contain cerebellar nuclear cells (MGI Ref ID J:46670)
- 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 (MGI Ref ID J:46670)
- abnormal cerebellar layer morphology
- abnormalities of the layers are more severe in the ectopic tissue (MGI Ref ID J:46670)
- abnormal cerebellum anterior lobe morphology
- irregular thickness of the anterior lobe (MGI Ref ID J:14268)
- abnormal cerebellum vermis lobule morphology
- smaller lobule V and less pronounced hemispheric part of lobule VI (MGI Ref ID J:46670)
- lobule VI does not appear to extend to the lateral aspect of the hemisphere (MGI Ref ID J:46670)
- lobule IX is reduced and appears to be joined to lobule VIII in the midline region (MGI Ref ID J:46670)
- the medial cerebellum usually has only 2 lobules rather than 3 as in wild-type mice (MGI Ref ID J:46670)
- small cerebellum (MGI Ref ID J:46670)
- increased inferior colliculus size
- appears to extend further posteriorly (MGI Ref ID J:46670)
- behavior/neurological phenotype
- abnormal gait
- swaying gait seen at 12 days of age (MGI Ref ID J:1827)
- abnormal posture
- ataxia
- head bobbing
- tend to bob up and down or side to side (MGI Ref ID J:46670)
- tremors
- slight (MGI Ref ID J:46670)
- growth/size phenotype
- postnatal growth retardation
- smaller than littermates at 12 days of age but catch up in size by 5 - 6 weeks of age (MGI Ref ID J:1827)
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:Unc5crcm related
Neurobiology Research
Ataxia (Movement) Defects
Cerebellar Defects
Receptor Defects
| 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 | ||
| Strain of Origin | old mutant of the mouse fancy | ||
| Gene Symbol and Name | a, nonagouti | ||
| Chromosome | 2 | ||
| Gene Common Name(s) | AGSW; AGTI; AGTIL; ASP; As; SHEP9; agouti; agouti signal protein; agouti suppressor; | ||
| Molecular Note | Characterization of this allele shows an insertion of DNA comprised of a 5.5kb virus-like element, VL30, into the first intron of the agouti gene. The VL30 element itself contains an additional 5.5 kb sequence, flanked by 526 bp of direct repeats. The host integration site is the same as for at-2Gso and Aw-38J and includes a duplication of four nucleotides of host DNA and a deletion of 2 bp from the end of each repeat. Northern analysis of mRNA from skin of homozygotes shows a smaller agouti message and levels 8 fold lower than found in wild-type. [MGI Ref ID J:16984] [MGI Ref ID J:24934] | ||
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Rakyan VK; Chong S; Champ ME; Cuthbert PC; Morgan HD; Luu KV; Whitelaw E. 2003. Transgenerational inheritance of epigenetic states at the murine Axin(Fu) allele occurs after maternal and paternal transmission. Proc Natl Acad Sci U S A 100(5):2538-43. [PubMed: 12601169] [MGI Ref ID J:82396]
Rice RH; Bradshaw KM; Durbin-Johnson BP; Rocke DM; Eigenheer RA; Phinney BS; Sundberg JP. 2012. Differentiating inbred mouse strains from each other and those with single gene mutations using hair proteomics. PLoS One 7(12):e51956. [PubMed: 23251662] [MGI Ref ID J:195664]
Rosenfeld CS; Sieli PT; Warzak DA; Ellersieck MR; Pennington KA; Roberts RM. 2013. Maternal exposure to bisphenol A and genistein has minimal effect on A(vy)/a offspring coat color but favors birth of agouti over nonagouti mice. Proc Natl Acad Sci U S A 110(2):537-42. [PubMed: 23267115] [MGI Ref ID J:193279]
Russell ES. 1948. A Quantitative Histological Study of the Pigment Found in the Coat Color Mutants of the House Mouse. II. Estimates of the Total Volume of Pigment. Genetics 33(3):228-36. [PubMed: 17247280] [MGI Ref ID J:148462]
Russell ES. 1946. A Quantitative Histological Study of the Pigment Found in the Coat-Color Mutants of the House Mouse. I. Variable Attributes of the Pigment Granules. Genetics 31(3):327-46. [PubMed: 17247200] [MGI Ref ID J:148463]
Russell ES. 1949. A Quantitative Histological Study of the Pigment Found in the Coat-Color Mutants of the House Mouse. IV. the Nature of the Effects of Genic Substitution in Five Major Allelic Series. Genetics 34(2):146-66. [PubMed: 17247308] [MGI Ref ID J:12958]
Russell LB. 1964. Genetic and Functional Mosaicism in the Mouse. In: The Role of the Chromosomes in Development. Academic Press, New York. [MGI Ref ID J:29504]
Russell LB; Cupp McDaniel MN; Woodiel FN,. 1963. Crossing over within the a "locus" of the mouse Genetics 48:907 Abstr. [MGI Ref ID J:174047]
SILVERS WK. 1958. An experimental approach to action of genes at the agouti locus in the mouse. III. Transplants of newborn Aw-, A-and at-skin to Ay-, Aw-, A-and aa hosts. J Exp Zool 137(1):189-96. [PubMed: 13563791] [MGI Ref ID J:13013]
Sakurai T; Ochiai H; Takeuchi T. 1975. Ultrastructural change of melanosomes associated with agouti pattern formation in mouse hair. Dev Biol 47(2):466-71. [PubMed: 1204945] [MGI Ref ID J:5606]
Silvers WK. 1979. The Coat Colors of Mice; A Model for Mammalian Gene Action and Interaction. In: The Coat Colors of Mice. Springer-Verlag, New York. [MGI Ref ID J:78801]
Soeller WC; Janson J; Hart SE; Parker JC; Carty MD; Stevenson RW; Kreutter DK; Butler PC. 1998. Islet amyloid-associated diabetes in obese A(vy)/a mice expressing human islet amyloid polypeptide. Diabetes 47(5):743-50. [PubMed: 9588445] [MGI Ref ID J:133694]
Suto J. 2008. Coincidence of loci for glucosuria and obesity in type 2 diabetes-prone KK-Ay mice. Med Sci Monit 14(2):CR65-74. [PubMed: 18227763] [MGI Ref ID J:131439]
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Animal Health Reports
Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.
| Pricing for USA, Canada and Mexico shipping destinations |
|
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2250.00 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.
Embryos
Price (US dollars $) Frozen Embryo $1600.00 Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
- Cryopreserved Embryos
Available to most shipping destinations1
This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.
1 Shipments cannot be made to Australia due to Australian government import restrictions.
2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.- Cryorecovery - Standard.
Progeny testing is not required.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 11 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
| Pricing for International shipping destinations |
|
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2925.00 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.
Embryos
Price (US dollars $) Frozen Embryo $2080.00 Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
- Cryopreserved Embryos
Available to most shipping destinations1
This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.
1 Shipments cannot be made to Australia due to Australian government import restrictions.
2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.- Cryorecovery - Standard.
Progeny testing is not required.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 11 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
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Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
| Control | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.
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Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.