『徳島大学 教育・研究者情報データベース (EDB)』---[学外] /
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種別 (必須): 学術論文 (審査論文) [継承]
言語 (必須): 英語 [継承]
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著者 (必須): 1. (英) Tominaga Kumiko (日) (読)
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2. (英) Matsuda Junko (日) (読)
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3. (英) Kido Makiko (日) (読)
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4. (英) Naito Etsuo (日) (読)
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5.横田 一郎
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6.樋田 一徳
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7.石村 和敬
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8. (英) Suzuki Kunihiko (日) (読)
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9.黒田 𣳾弘
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題名 (必須): (英) Genetic Background Markedly Influences Vulnerability of the Hippocampal Neuronal Organization in the ''Twitcher'' Mouse Model of Globoid Cell Leukodystrophy  (日)    [継承]
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要約 (任意): (英) The twitcher mouse is well known as a naturally occurring authentic mouse model of human globoid cell leukodystrophy (GLD; Krabbe disease) due to genetic deficiency of lysosomal galactosylceramidase. The twitcher mice used most commonly are on the C57BL/6J background. We generated twitcher mice that were on the mixed background of C57BL/6J and 129SvEv, the standard strain for production of targeted mutations. Twitcher mice on the mixed background were smaller and had a shorter lifespan than were those on the C57BL/6J background. Many twitcher mice on the mixed background developed generalized seizures around 30 days that were never seen in twitcher mice on the C57BL/6J background. Neuropathologically, although the degree of the typical demyelination with infiltration of macrophages was similar in the central and peripheral nervous systems, in both strains, marked neuronal cell death was observed only in twitcher mice on the mixed background. In the hippocampus, the neuronal cell death occurred prominently in the CA3 region in contrast to the relatively well-preserved CA1 and CA2 areas. This neuropathology has never been seen in twitcher mice on the C57BL/6J background. Biochemically, the brain of twitcher mice on the mixed background showed much greater accumulation of lactosylceramide. Genetic background must be carefully taken into consideration when phenotype of mutant mice is evaluated, particularly because most targeted mutants are initially on a mixed genetic background and gradually moved to a pure background. These findings also suggest an intriguing possibility of important function of some sphingolipids in the hippocampal neuronal organization and maintenance.  (日)    [継承]
キーワード (推奨): 1. (英) Krabbe disease (日) (読) [継承]
2. (英) hippocampus (日) (読) [継承]
3. (英) neuronal cell death (日) (読) [継承]
4. (英) sphingolipids (日) (読) [継承]
5. (英) lactosylceramide (日) (読) [継承]
発行所 (推奨): (英) Wiley Interscience (日) (読) [継承]
誌名 (必須): Journal of Neuroscience Research (Wiley Interscience)
(pISSN: 0360-4012, eISSN: 1097-4547)

ISSN (任意): 0360-4012
ISSN: 0360-4012 (pISSN: 0360-4012, eISSN: 1097-4547)
Title: Journal of neuroscience research
Title(ISO): J Neurosci Res
Publisher: Wiley
 (NLM Catalog  (Wiley  (Scopus  (CrossRef (Scopus information is found. [need login])
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(必須): 77 [継承]
(必須): 4 [継承]
(必須): 507 516 [継承]
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年月日 (必須): 西暦 2004年 8月 15日 (平成 16年 8月 15日) [継承]
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DOI (任意): 10.1002/jnr.20190    (→Scopusで検索) [継承]
PMID (任意): 15264220    (→Scopusで検索) [継承]
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WOS (任意): 000223020800005 [継承]
Scopus (任意): 2-s2.0-3442886765 [継承]
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備考 (任意): 1.(英) Article.Affiliation: Department of Pediatrics, University of Tokushima School of Medicine, Tokushima, Japan.  (日)    [継承]
2.(英) Article.PublicationTypeList.PublicationType: Journal Article  (日)    [継承]
3.(英) Article.PublicationTypeList.PublicationType: Research Support, Non-U.S. Gov't  (日)    [継承]

標準的な表示

和文冊子 ● Kumiko Tominaga, Junko Matsuda, Makiko Kido, Etsuo Naito, Ichiro Yokota, Kazunori Toida, Kazunori Ishimura, Kunihiko Suzuki and Yasuhiro Kuroda : Genetic Background Markedly Influences Vulnerability of the Hippocampal Neuronal Organization in the ''Twitcher'' Mouse Model of Globoid Cell Leukodystrophy, Journal of Neuroscience Research, Vol.77, No.4, 507-516, 2004.
欧文冊子 ● Kumiko Tominaga, Junko Matsuda, Makiko Kido, Etsuo Naito, Ichiro Yokota, Kazunori Toida, Kazunori Ishimura, Kunihiko Suzuki and Yasuhiro Kuroda : Genetic Background Markedly Influences Vulnerability of the Hippocampal Neuronal Organization in the ''Twitcher'' Mouse Model of Globoid Cell Leukodystrophy, Journal of Neuroscience Research, Vol.77, No.4, 507-516, 2004.

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