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 development愛知県心身障害者コロニー発達障害研究所・米澤　敏先生。KYF/MsIdr-Jsr系統に生じた自然突然変異。
Strain descriptionCdh23遺伝子に生じたbus突然変異。ホモマウスは内耳有毛神経細胞変性を起こす。旋回、頭振の行動異常、内耳性難聴を呈する。ホモ雌は繁殖が可能なものの、育仔は困難。bus系統 (RBRC00073) 、コントロール (正常) 系統 (RBRC00072) 。
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:K囮検査
2025/08/04Room:4-BRack:K囮検査
2025/05/26Room:4-BRack:K囮検査
2025/02/25Room:4-BRack:K囮検査
2024/11/25Room:4-BRack:K囮検査
2024/08/26Room:4-BRack:K囮検査
2024/05/27Room:4-BRack:K囮検査

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

    供与核酸
    Research applicationNeurobiology Research
    Sensorineural Research
    提供条件条件を付加しない。
    Depositor米澤　敏（愛知県心身障害者コロニー発達障害研究所）
    Strain Status凍結胚のアイコン凍結胚
    凍結精子のアイコン凍結精子
    Strain Availability凍結胚より作出したマウスを2~4ヶ月以内に提供可能
    凍結精子を1ヶ月以内に提供可能
    凍結胚を1ヶ月以内に提供可能
    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