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Former Names STOCK rd8 (Changed: 15-DEC-04 ) Type Mutant Stock; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Mating System Homozygote x Homozygote (Female x Male) 01-MAR-06 Species laboratory mouse Generation N3F12+ (09-OCT-03) Appearance
black
Related Genotype: a/aImportant Note
The C57BL/6J background strain is homozygous for the age related hearing loss mutation Cdh23ahl, which on this background results in progressive hearing loss with onset after 10 months of age.Development
The Catb (catalase 1 null) mutation was identified in a screen for low catalase activity by Feinstein et al. (1964, 1966) at Argonne National Laboratory of offpring of (C3H x 101) male mice irradiated at Oak Ridge National Laboratory (600 R in fractionated exposures), then crossed to females of the Oak Ridge "multiple recessive testing stock" derived from a cross of NB mice (aabbcch p/cch p d se/d se) and mice of a non-inbred stock homozygous for three of the same recessive mutations, as well as for s, from which mice homozygous for all seven mutations were bred and maintained afterward by random breeding (Russell 1951). Putative heterozygotes for catalase deficiency were crossed to normal mice (not clearly identified, but presumably unirradiated progeny of (101 x C3H)F1 X ORNL testing stock), and offspring exhibiting low catalase activity were backcrossed to the heterozygous parent (Feinstein et al. 1966).Catb was subsequently transferred by backcrossing for eight generations onto C3H/HeAnl, a line carrying mouse mammary tumor virus (MMTV) and another line (C3Hf) free of the virus (Feinstein et al. 1978). The Jackson Laboratory received C3HfB/Ga-Catb from Allen H. Gates, then at the University of Rochester, in 1982.Although all C3H strains carry the rd1 (retinal degeneration 1) mutation at Pde6b, the gene encoding the beta subunit of phosphodiesterase 1, retinas of these mice exhibited a peculiar, granular appearance distinct from the usual Pde6brd1 phenotype. F2 progeny of a cross of C3HfB/Ga-Catb and C57BL/6J mice exhibited three retinal phenotypes: 1) normal; 2) patches of pigment deposition and large, diffuse pigmented regions typical Pde6brd1 homozygotes; and 3) small, discrete, light-colored dots throughout the fundus. Mice exhibiting the last phenotype were intercrossed among themselves to produce a stock homozygous for Mfrprd6 and for the wild-type allele at Pde6b. During this process, a separate mutation with a distinct map position was identified and characterized as Crb1rd8. STOCK Crb1rd8 Mfrprd6/J (stock #004299) is homozygous for both Mfrprd6 and Crb1rd8 on this mixed genetic background that derived in part from C57BL/6J, C3H, 101, and a linkage testing stock derived from non-inbred stocks. In order to isolate Mfrprd6 and Crb1rd8 this strain was backcrossed to C57BL/6J, and selection for either Mfrprd6 or Crb1rd8 yielded B6.C3Ga-Mfrprd6/J (stock #003684) and STOCK Crb1rd8/J (stock #003392) respectively.
| Control | ||
|---|---|---|
| 000664 C57BL/6J | (approximate) | |
| C57BL/6J (Stock Number 000664) is only an approximate control for this stock, whose genetic background is ~87.5% C57BL/6J and ~12.5% C3HfB/Ga. | ||
| Considerations for Choosing Controls | ||
Strains carrying Cdh23ahl allele
001137 129P1/ReJ 000690 129P3/J 000691 129X1/SvJ 000646 A/J 000647 A/WySnJ 003070 ALR/LtJ 003072 ALS/LtJ 004502 B6;AKR-Lxl2/GrsrJ 001026 BALB/cByJ 000653 BUB/BnJ 005494 C3.129S1(B6)-Grm1rcw/J 000664 C57BL/6J 004764 C57BL/6J-Cdh23v-8J/J 003129 C57BL/6J-Epha4rb-2J/GrsrJ 004820 C57BL/6J-Kcne12J/J 004703 C57BL/6J-Kcnq2Nmf134/J 004811 C57BL/6J-nmf110/J 004812 C57BL/6J-nmf111/J 004747 C57BL/6J-nmf118/J 004656 C57BL/6J-nmf88/J 004391 C57BL/6J-Chr 13A/J/NaJ 004385 C57BL/6J-Chr 7A/J/NaJ 000662 C57BLKS/J 000667 C57BR/cdJ 000668 C57L/J 000669 C58/J 000657 CE/J 000670 DBA/1J 001140 DBA/1LacJ 000671 DBA/2J 007048 DBA/2J-Gpnmb+/SjJ 002106 KK/HlJ 000675 LG/J 000676 LP/J 000677 MA/MyJ 001976 NOD/ShiLtJ 002050 NOR/LtJ 000679 P/J 002747 SENCARB/PtJ 002335 SKH2/J View Strains carrying Cdh23ahl (40 strains)
Strains carrying Crb1rd8 allele
004852 B6;129-Crb1rd8/J View Strains carrying Crb1rd8 (1 strain)
Strains carrying other alleles of Cdh23
002756 B6.CAST-Cdh23Ahl+/Kjn 002432 B6J x B6.C-H2-Kbm1/ByJ-Cdh23v-J/J 002552 C57BL/6J-Cdh23v-2J/J 004764 C57BL/6J-Cdh23v-8J/J 004819 C57BL/6J-Cdh23v-9J/J 005016 CByJ;B6-Cdh23v-10J/J 000275 V/LeJ View Strains carrying other alleles of Cdh23 (7 strains)
View Mammalian Phenotype Terms
Mammalian Phenotype Terms
assigned by genotype
Crb1rd8/Crb1rd8
STOCK Crb1rd8/J
- vision/eye phenotype
- abnormal ocular fundus morphology (MGI Ref ID J:85459)
- mice exhibit large, irregularly shaped spots concentrated in the inferior nasal quadrant of the fundus as early as week 3 that correspond to retinal folds and pseudorosettes
- abnormal retina morphology (MGI Ref ID J:85459)
- retinal thinning in both the inner and outer segment is observed
- the external limiting membrane in the retina is fragmented and disorganized at 2 weeks and is more severe at 4 weeks
- abnormal Muller cell morphology (MGI Ref ID J:85459)
- Muller cells exhibit increased electron density and cytoplasmic strands that are more easily traced through the plexiform layer than in wild-type mice
- abnormal retinal photoreceptor morphology (MGI Ref ID J:85459)
- the photoreceptor lamellae breaks down
- abnormal photoreceptor inner segment morphology (MGI Ref ID J:85459)
- the photoreceptor inner segments lose their orderly arrangement
- by 5 months the inner segment approaches the retinal pigment epithelium
- swelling occurs in portions of the inner segments
- short photoreceptor inner segment (MGI Ref ID J:85459)
- at 4 weeks the photoreceptor inner segments are 25% shorter
- decreased retinal photoreceptor cell number (MGI Ref ID J:85459)
- in the region of focal degeneration photoreceptors are lost
- photoreceptor outer segment degeneration (MGI Ref ID J:85459)
- by week 10 the outer segment begins to fragment
- by 5 months only a few outer segment fragments remain
- short photoreceptor outer segment (MGI Ref ID J:85459)
- the photoreceptor outer segments are shortened
- thin retinal outer nuclear layer (MGI Ref ID J:85459)
- in older mice, the retinal outer nuclear layer is reduced to a single row of nuclei
- nervous system phenotype
- abnormal Muller cell morphology (MGI Ref ID J:85459)
- Muller cells exhibit increased electron density and cytoplasmic strands that are more easily traced through the plexiform layer than in wild-type mice
- abnormal retinal photoreceptor morphology (MGI Ref ID J:85459)
- the photoreceptor lamellae breaks down
- abnormal photoreceptor inner segment morphology (MGI Ref ID J:85459)
- the photoreceptor inner segments lose their orderly arrangement
- by 5 months the inner segment approaches the retinal pigment epithelium
- swelling occurs in portions of the inner segments
- short photoreceptor inner segment (MGI Ref ID J:85459)
- at 4 weeks the photoreceptor inner segments are 25% shorter
- decreased retinal photoreceptor cell number (MGI Ref ID J:85459)
- in the region of focal degeneration photoreceptors are lost
- photoreceptor outer segment degeneration (MGI Ref ID J:85459)
- by week 10 the outer segment begins to fragment
- by 5 months only a few outer segment fragments remain
- short photoreceptor outer segment (MGI Ref ID J:85459)
- the photoreceptor outer segments are shortened
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Crb1rd8/Crb1rd8
involves: C3HfB6/Ga * C57BL/6 * CAST/EiJ
- vision/eye phenotype
- abnormal retina morphology (MGI Ref ID J:85459)
- 19% of mice do not display the spotting phenotype on a CAST/EiJ mixed background
Crb1rd8/Crb1rd8
B6.Cg-Crb1rd8
- vision/eye phenotype
- *normal* vision/eye phenotype (MGI Ref ID J:85459)
- mice no longer display the spotting phenotype when backcrossed from a CAST/EiJ background onto a C57BL/6 background
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:Cdh23ahl related
Crb1rd8 relatedNeurobiology Research
Vestibular and Hearing Defects
Age related hearing loss
Sensorineural Research
Vestibular and Hearing Defects
Age related hearing loss
Cell Biology Research
Defects in Cell Adhesion Molecules
Developmental Biology Research
Eye Defects
Mouse/Human Gene Homologs
Retinitis pigmentosa 12; Leber congenital amaurosis (CLA)
Sensorineural Research
Retinal Degeneration
| Allele Symbol | Crb1rd8 | ||
|---|---|---|---|
| Allele Name | retinal degeneration 8 | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | nmf144; | ||
| Strain of Origin | C57BL/6J | ||
| Gene Symbol and Name | Crb1, crumbs homolog 1 (Drosophila) | ||
| Chromosome | 1 | ||
| Gene Common Name(s) | 7530426H14Rik; A930008G09Rik; LCA8; RIKEN cDNA 7530426H14 gene; RIKEN cDNA A930008G09 gene; RP12; | ||
| Molecular Note | The mutation in the rd8 mouse has been identified as a single base deletion in the Crb1 gene. This deletion causes a frame shift and a premature stop codon that truncates the transmembrane and cytoplasmic domain of the protein. [MGI Ref ID J:85459] | ||
| Allele Symbol | Cdh23ahl | ||
| Allele Name | age related hearing loss 1 | ||
| Allele Type | QTL | ||
| Common Name(s) | Cdh23753A; mdfw; | ||
This strain will not have a genotyping protocol or one is not currently available.
Helpful Links
Genotyping resources and troubleshooting
Mehalow AK; Kameya S; Smith RS; Hawes NL; Denegre JM; Young JA; Bechtold L; Haider NB; Tepass U; Heckenlively JR; Chang B; Naggert JK; Nishina PM. 2003. CRB1 is essential for external limiting membrane integrity and photoreceptor morphogenesis in the mammalian retina. Hum Mol Genet 12(17):2179-89. [PubMed: 12915475] [MGI Ref ID J:85459]
Crb1rd8 relatedChang B; Hawes NL; Hurd RE; Davisson MT; Nusinowitz S; Heckenlively JR. 2002. Retinal degeneration mutants in the mouse. Vision Res 42(4):517-25. [PubMed: 11853768] [MGI Ref ID J:75095]
Animal Health Reports
Room Number A1
Colony Maintenance
Mating System Homozygote x Homozygote (Female x Male) 01-MAR-06
| Pricing for USA, Canada and Mexico shipping destinations |
|
Weeks of Age Price (US dollars $) Gender Genotypes Provided Individual Mouse $107.90 Female or Male Homozygous for Crb1rd8
Pairs /Price (US dollars $) Pair Genotype $215.80 Homozygous for Crb1rd8 x Homozygous for Crb1rd8
| Pricing for International shipping destinations |
|
Weeks of Age Price (US dollars $) Gender Genotypes Provided Individual Mouse $140.30 Female or Male Homozygous for Crb1rd8
Pairs /Price (US dollars $) Pair Genotype $280.60 Homozygous for Crb1rd8 x Homozygous for Crb1rd8
| Standard Supply | Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of approximately nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within two business days following order placement. |
|---|---|
| Supply Notes |
|
| Important Note | |
| The C57BL/6J background strain is homozygous for the age related hearing loss mutation Cdh23ahl, which on this background results in progressive hearing loss with onset after 10 months of age. | |
| Control | ||
|---|---|---|
| 000664 C57BL/6J | (approximate) | |
| C57BL/6J (Stock Number 000664) is only an approximate control for this stock, whose genetic background is ~87.5% C57BL/6J and ~12.5% C3HfB/Ga. | ||
| 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. | ||
Purchasing Information
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Contact Information
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Fax: 1-207-288-6150
Technical Support Email Form
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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