Strain Data Sheet

RBRC00073

Strain Information

Image
BRC No.RBRC00073
TypeSpontaneous Mutation
SpeciesMus musculus
Strain nameBUS/Idr-bus
Former Common nameBUS/Idr-bus
H-2 Haplotype
ES Cell line
Background strainKYF
Appearancebrown agouti [A/A b/b C/C]
Strain developmentDeveloped by Satoshi Yonezawa, Institute for Developmental Research, Japan. Spontaneous mutation originally arosed in KYF/MsIdr-Jsr strain.
Strain descriptionbustling spontaneous mutation arose in Cdh23 gene. Homozygous mutant mice exhibit behavioral abnormalities such as hyperactivity, circling, head tossing and head tilting with vestibulocochlear defects. Viable and fertile, but it is difficult for homozygous female to nurse pups.
Colony maintenanceHeterozygote (female) x Homozygote (male) [or Crossing to BUS/Idr-+/+(RBRC00072)]
References
Vestibulocochlear defects and effects of deuterium oxide in mutant bustling (BUS) mice.
H Otani, K Moriyama, S Yonezawa, R Shoji, O Tanaka
Acta Otolaryngol. Suppl., 519, 286-293 (1995). 7610888

Cochlear histopathology of the mutant bustling mouse, BUS/Idr.
S Yonezawa, Y Nodasaka, T Kamada, S C Fujita, K Kato, Y Yamada, N Ogasawara, R Shoji
Acta Otolaryngol. (Stockh), 116, 409-416 (1996). 8790740

Degenerative hairlets on the vestibular sensory cells in mutant bustling (BUS/Idr) mice.
K Moriyama, R Hashimoto, A Hanai, N Yoshizaki, S Yonezawa, H Otani
Acta Otolaryngol., 117, 20-24 (1997). 9039475

Chromosomal localization of a gene responsible for vestibulocochlear defects of BUS/Idr mice: identification as an allele of waltzer.
S Yonezawa, A Yoshiki, A Hanai, T Matsuzaki, J Matsushima, T Kamada, M Kusakabe
Hear. Res., 134, 116-122 (1999). 10452381

Fates of Cdh23/CDH23 with mutations affecting the cytoplasmic region.
Satoshi Yonezawa, Norio Yoshizaki, Takashi Kageyama, Takayuki Takahashi, Mamoru Sano, Yoshihito Tokita, Shigeo Masaki, Yutaka Inaguma, Atsuko Hanai, Nobuhiko Sakurai, Atsushi Yoshiki, Moriaki Kusakabe, Akihiko Moriyama, Atsuo Nakayama
Human Mutation, 27, 88-97 (2006). 16281288

Health Report

Examination Date / Room / Rack2025/10/20Room:4-BRack:KSentinel mouse program
2025/08/04Room:4-BRack:KSentinel mouse program
2025/05/26Room:4-BRack:KSentinel mouse program
2025/02/25Room:4-BRack:KSentinel mouse program
2024/11/25Room:4-BRack:KSentinel mouse program
2024/08/26Room:4-BRack:KSentinel mouse program
2024/05/27Room:4-BRack:KSentinel mouse program

Gene

Gene SymbolGene NameChr.Allele SymbolAllele NameCommon NamesPromoterDiseases Related to This Gene
Cdh23
MGI:1890219		cadherin related 23 (otocadherin)10Cdh23<v-bus>
MGI:1856681		bustling
  • autosomal recessive nonsyndromic hearing loss 12(DisGeNET, MedGEN)

  • pituitary adenoma 5, multiple types(MedGEN)

  • Usher syndrome type 1D(DisGeNET, MedGEN)

    • Phenotype

    Phenotype annotation from literatures by Mammalian phenotype ontology
  • abnormal cochlea morphology (MP:0000031)

  • abnormal cochlear ganglion morphology (MP:0002855)

  • abnormal cochlear hair cell morphology (MP:0002622)

  • abnormal cochlear sensory epithelium morphology (MP:0003308)

  • abnormal inner hair cell stereociliary bundle morphology (MP:0004532)
    • more 12 phenotypes
  • abnormal maternal nurturing (MP:0001386)

  • abnormal organ of Corti morphology (MP:0000042)

  • abnormal orientation of inner hair cell stereociliary bundles (MP:0004492)

  • abnormal orientation of outer hair cell stereociliary bundles (MP:0004491)

  • abnormal outer hair cell stereociliary bundle morphology (MP:0004527)

  • abnormal startle reflex (MP:0001486)

  • absent startle reflex (MP:0006359)

  • circling (MP:0001394)

  • decreased cochlear hair cell number (MP:0004408)

  • head tilt (MP:0005191)

  • head tossing (MP:0005307)

  • hyperactivity (MP:0001399)
    • Detailed phenotype data

    Ordering Information

    Donor DNA
    Research applicationNeurobiology Research
    Sensorineural Research
    Specific Term and ConditionsNo specific terms and conditions. (The DEPOSITOR waives its own rights under any patents, intellectual property, or other proprietary rights with respect to the results to be obtained by use of the BIOLOGICAL RESOURCE.)
    DepositorSatoshi Yonezawa (Aichi Human Service Center)
    Strain Statusan icon for Frozen embryosFrozen embryos
    an icon for Frozen spermFrozen sperm
    Strain AvailabilityRecovered litters from cryopreserved embryos (2 to 4 months)
    Cryopreserved sperm (within 1 month)
    Cryopreserved embryos (within 1 month)
    Additional Info.Necessary documents for ordering:
    1. Order form (Japanese / English)
    2. Category I MTA: MTA for distribution with RIKEN BRC (Japanese / English)

    BRC mice in Publications

    No Data