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Histological analysis of the brain in Dystonin-deficient mice

  • P3-304
  • 堀江 正男 / Masao Horie:1 渡辺 啓介 / Keisuke Watanabe:1 佐野 裕美 / Hiromi Sano:2 梨本 淳一郎 / Jun-ichiro Nashimoto:1 知見 聡美 / Satomi Chiken:2 南部 篤 / Atsushi Nambu:2 小野 勝彦 / Katsuhiko Ono:3 池中 一裕 / Kazuhiro Ikenaka:4 柿田 明美 / Akiyoshi Kakita:5 竹林 浩秀 / Hirohide Takebayashi:1 
  • 1:新潟大学 / Div. Neurobiol. & Anat., Dept. Sensory & Integr. Med., Niigata Univ., Grad. Sch. of Med. & Dent. Scis. 2:生理学研究所 生体システム研究部門 / Div of System Neurophysiol., NIPS, Okazaki, Japan 3:京都府立医科大学大学院 医学研究科 神経発生生物学 / Dept of Biol, Kyoto Pref Univ of Med 4:生理学研究所 分子神経生理学部門 / Div of Neurobiol and Bioinfomatics, NIPS, Okazaki, Japan 5:新潟大学脳研究所 脳疾患標本資源解析学分野 / Dept of Pathol. Neurosci., BRI, Niigata Univ 

Dystonia musculorum (dt), is an inherited mouse neuropathy characterized by progressive motor disorders. Dystonin (Dst) is a causative gene for dt mice. Although degeneration of the peripheral nervous system (PNS) during early postnatal stage is well-recognized phenotype in dt mice, histological appearance in the central nervous system (CNS) responsible for motor disorders are still unclear. We generated a novel Dst gene trap mice, DstGt, in which actin binding domain-containing isoforms are disrupted. Homozygous mice showed typical dt phenotypes with progressive neurological symptoms. The gene trap allele encodes for a mutant Dst-LacZ fusion protein which is detected by X-gal staining with high sensitivity. We observed severe motor disorders in their limbs and twisted postures. Electorophysiological study showed abnormal co-contractions of agonist and antagonist muscle in DstGt homozygotes. In histological aspects, abnormal neurofilament immunoreactivity was found in both somatosensory pathway and motor-related medullary reticular nucleus which showed high Dst expression. These results raise the possibility that cell-autonomous primary CNS defects contribute to dt phenotype.

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