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  1. 010 学術雑誌論文
  2. 蟻川, 謙太郎 / ARIKAWA, Kentaro

Defective phototransductive disk membrane morphogenesis in transgenic mice expressing opsin with a mutated N-terminal domain

https://ir.soken.ac.jp/records/3275
https://ir.soken.ac.jp/records/3275
247835f6-835f-40ad-bbc6-c70ff40d199e
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2589.full.pdf 2589.full (659.8 kB)
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Item type 学術雑誌論文 / Journal Article(1)
公開日 2012-12-12
タイトル
タイトル Defective phototransductive disk membrane morphogenesis in transgenic mice expressing opsin with a mutated N-terminal domain
タイトル
タイトル Defective phototransductive disk membrane morphogenesis in transgenic mice expressing opsin with a mutated N-terminal domain
言語 en
言語
言語 eng
資源タイプ
資源タイプ識別子 http://purl.org/coar/resource_type/c_6501
資源タイプ journal article
著者 LIU, Xinran

× LIU, Xinran

LIU, Xinran

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WU, Ting-Huai

× WU, Ting-Huai

WU, Ting-Huai

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STOWE, Sally

× STOWE, Sally

STOWE, Sally

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MATSUSHITA, Atsuko

× MATSUSHITA, Atsuko

MATSUSHITA, Atsuko

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ARIKAWA, Kentaro

× ARIKAWA, Kentaro

ARIKAWA, Kentaro

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NAASH, Muna I

× NAASH, Muna I

NAASH, Muna I

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WILLIAMS, David S

× WILLIAMS, David S

WILLIAMS, David S

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著者別名 蟻川, 謙太郎

× 蟻川, 謙太郎

蟻川, 謙太郎

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内容記述タイプ Abstract
内容記述 Retinitis pigmentosa is a heterogeneous group of inherited retinal disorders in which the photoreceptor cells degenerate. A line of transgenic mice expresses a mutant opsin gene that encodes three missense mutations near the amino terminus, including P23H, which is the basis for a common form of dominant retinitis pigmentosa. By studying the photoreceptor cells of these mice and their normal littermates, we found that: (1) opsin was routed correctly, (2) the concentration of opsin in the disk membranes appeared normal by freeze fracture analysis, (3) the amount of disk membrane shedding was normal, but (4) the basal disks of the outer segments were disorganized, indicating defective disk membrane morphogenesis. Defective disk membrane morphogenesis appears to result in the formation of fewer mature disks, thus accounting for observed gradual shortening of the photoreceptor outer segments with age. We suggest that abnormal disk membrane morphogenesis is the primary cellular defect that leads to blindness, and that it arises from the inability of nascent disk membranes, containing normal and mutant opsin, to interact normally with each other.
書誌情報 Journal of Cell Science
en : Journal of Cell Science

巻 110, 号 20, p. 2589-2597, 発行日 1997-10-15
出版者
出版者 The Company of Biologists Ltd
ISSN
収録物識別子タイプ ISSN
収録物識別子 00219533
PubMed番号
関連タイプ isIdenticalTo
識別子タイプ PMID
関連識別子 9372448
権利
権利情報 © 1997 by Company of Biologists
関連サイト
識別子タイプ URI
関連識別子 http://www.biologists.com/copyright_permissions.html
関連名称 Copyright
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内容記述タイプ Other
内容記述 application/pdf
著者版フラグ
出版タイプ VoR
出版タイプResource http://purl.org/coar/version/c_970fb48d4fbd8a85
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