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Simplified drug efficacy evaluation system for vasopressin neurodegenerative disease using mouse disease-specific induced pluripotent stem cells.
Miwata, Tsutomu; Suga, Hidetaka; Mitsumoto, Kazuki; Zhang, Jun; Hamada, Yoshimasa; Sakakibara, Mayu; Soen, Mika; Ozaki, Hajime; Asano, Tomoyoshi; Miyata, Takashi; Kawaguchi, Yohei; Yasuda, Yoshinori; Kobayashi, Tomoko; Sugiyama, Mariko; Onoue, Takeshi; Hagiwara, Daisuke; Iwama, Shintaro; Oyadomari, Seiichi; Arima, Hiroshi.
Afiliación
  • Miwata T; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Suga H; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan. Electronic address: sugahide@med.nagoya-u.ac.jp.
  • Mitsumoto K; Department of Endocrinology and Diabetes, Gifu Prefectural Tajimi Hospital, Tajimi, Japan.
  • Zhang J; Division of Molecular Biology, Institute of Advanced Medical Sciences, Tokushima University, Tokushima, Japan.
  • Hamada Y; Division of Molecular Biology, Institute of Advanced Medical Sciences, Tokushima University, Tokushima, Japan.
  • Sakakibara M; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Soen M; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Ozaki H; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Asano T; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Miyata T; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Kawaguchi Y; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Yasuda Y; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Kobayashi T; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Sugiyama M; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Onoue T; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Hagiwara D; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Iwama S; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
  • Oyadomari S; Division of Molecular Biology, Institute of Advanced Medical Sciences, Tokushima University, Tokushima, Japan.
  • Arima H; Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Peptides ; 173: 171151, 2024 Mar.
Article en En | MEDLINE | ID: mdl-38215943
ABSTRACT
Familial neurohypophyseal diabetes insipidus (FNDI) is a degenerative disorder in which vasopressin-secreting neurons degenerate over time due to the production of mutant proteins. We have demonstrated therapeutic effects of chemical chaperones in an FNDI mouse model, but the complexity and length of this evaluation were problematic. In this study, we established disease-specific mouse induced pluripotent stem cells (iPSCs) from FNDI-model mice and differentiated vasopressin neurons that produced mutant proteins. Fluorescence immunostaining showed that chemical chaperones appeared to protect vasopressin neurons generated from iPSCs derived from FNDI-model mice. Although KCL stimulation released vasopressin hormone from vasopressin neurons generated from FNDI-derived iPSCs, vasopressin hormone levels did not differ significantly between baseline and chaperone-added culture. Semi-quantification of vasopressin carrier protein and mutant protein volumes in vasopressin neurons confirmed that chaperones exerted a therapeutic effect. This research provides fundamental technology for creating in vitro disease models using human iPSCs and can be applied to therapeutic evaluation of various degenerative diseases that produce abnormal proteins.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades Neurodegenerativas / Diabetes Insípida Neurogénica / Células Madre Pluripotentes Inducidas Límite: Animals / Humans Idioma: En Revista: Peptides Año: 2024 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades Neurodegenerativas / Diabetes Insípida Neurogénica / Células Madre Pluripotentes Inducidas Límite: Animals / Humans Idioma: En Revista: Peptides Año: 2024 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Estados Unidos