| 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
WU, Ting-Huai
STOWE, Sally
MATSUSHITA, Atsuko
ARIKAWA, Kentaro
NAASH, Muna I
WILLIAMS, David S
|
| 著者別名 |
蟻川, 謙太郎
|
| 抄録 |
|
|
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 |
| フォーマット |
|
|
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 |
2589.full (659.8 kB)
.png)
