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Apoptosis of type I spiral ganglion neuron cells in Otof-mutant mice.
Tsuzuki, Nobuyoshi; Namba, Kazunori; Saegusa, Chika; Mutai, Hideki; Nishiyama, Takanori; Oishi, Naoki; Matsunaga, Tatsuo; Fujioka, Masato; Ozawa, Hiroyuki.
Afiliación
  • Tsuzuki N; Department of Otolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35, Shinanomachi, Shinjuku, Tokyo 160-8582, Japan; Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, 2-5-1, Higashigaoka, Meguro
  • Namba K; Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, 2-5-1, Higashigaoka, Meguro, Tokyo 152-8902, Japan.
  • Saegusa C; Department of Otolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35, Shinanomachi, Shinjuku, Tokyo 160-8582, Japan; Department of Molecular Genetics, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374, Japan. Electronic address:
  • Mutai H; Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, 2-5-1, Higashigaoka, Meguro, Tokyo 152-8902, Japan. Electronic address: hideki.mutai@kankakuki.jp.
  • Nishiyama T; Department of Otolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35, Shinanomachi, Shinjuku, Tokyo 160-8582, Japan.
  • Oishi N; Department of Otolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35, Shinanomachi, Shinjuku, Tokyo 160-8582, Japan. Electronic address: oishin@keio.jp.
  • Matsunaga T; Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, 2-5-1, Higashigaoka, Meguro, Tokyo 152-8902, Japan; Department of Otolaryngology, National Hospital Organization Tokyo Medical Center, 2-5-1, Higashigaoka, Meguro, Tok
  • Fujioka M; Department of Otolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35, Shinanomachi, Shinjuku, Tokyo 160-8582, Japan; Department of Molecular Genetics, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374, Japan; Clinical and Transl
  • Ozawa H; Department of Otolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35, Shinanomachi, Shinjuku, Tokyo 160-8582, Japan.
Neurosci Lett ; 803: 137178, 2023 04 23.
Article en En | MEDLINE | ID: mdl-36914046
Otof, which encodes otoferlin, knockout mice are considered model mice for auditory neuropathy spectrum disorder, which is characterized by an absent auditory brainstem response (ABR) despite preserved distortion product otoacoustic emission (DPOAE). Although otoferlin-deficient mice lack neurotransmitter release at the inner hair cell (IHC) synapse, it remains unclear how the Otof mutation affects spiral ganglions. Thus, we used Otof-mutant mice carrying the Otoftm1a(KOMP)Wtsi allele (Otoftm1a) and analyzed spiral ganglion neurons (SGNs) in Otoftm1a/tm1a mice by immunolabeling type Ⅰ SGNs (SGN-Ⅰ) and type II SGNs (SGN-II). We also examined apoptotic cells in SGNs. Four-week-old Otoftm1a/tm1a mice had an absent ABR but normal DPOAEs. The number of SGNs was significantly lower in Otoftm1a/tm1a mice on postnatal day 7 (P7), P14, and P28 compared with that of wild-type mice. Moreover, significantly more apoptotic SGNs were observed in Otoftm1a/tm1a mice than in wild-type mice on P7, P14, and P28. SGN-IIs were not significantly reduced in Otoftm1a/tm1a mice on P7, P14, and P28. No apoptotic SGN-IIs were observed under our experimental conditions. In summary, Otoftm1a/tm1a mice showed a reduction in SGNs accompanied by apoptosis of SGN-Ⅰs even before the onset of hearing. We speculate that the reduction in SGNs with apoptosis is a secondary defect caused by a lack of otoferlin in IHCs. Appropriate glutamatergic synaptic inputs may be important for the survival of SGNs.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Ganglio Espiral de la Cóclea / Neuronas Límite: Animals Idioma: En Revista: Neurosci Lett Año: 2023 Tipo del documento: Article Pais de publicación: Irlanda
Texto completo
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Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Ganglio Espiral de la Cóclea / Neuronas Límite: Animals Idioma: En Revista: Neurosci Lett Año: 2023 Tipo del documento: Article Pais de publicación: Irlanda