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  1. C100 医学部/医学系研究科
  2. C100a 雑誌掲載論文
  3. 学術雑誌

Corneocyte lipid envelope (CLE), the key structure for skin barrier function and ichthyosis pathogenesis

http://hdl.handle.net/2237/27314
http://hdl.handle.net/2237/27314
ec0a13a0-60ed-4693-9ada-1f4d9ef5e125
名前 / ファイル ライセンス アクション
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Akiyama_M_J_Dermatol_Sci_Figure1.jpg Akiyama_M_J_Dermatol_Sci_Figure1.jpg ファイル公開日:2018/10/01 (1.8 MB)
Akiyama_M_J_Dermatol_Sci_Table1.pdf Akiyama_M_J_Dermatol_Sci_Table1.pdf ファイル公開日:2018/10/01 (20.4 kB)
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Item type 学術雑誌論文 / Journal Article(1)
公開日 2018-01-30
タイトル
タイトル Corneocyte lipid envelope (CLE), the key structure for skin barrier function and ichthyosis pathogenesis
言語 en
著者 Akiyama, Masashi

× Akiyama, Masashi

WEKO 74862

en Akiyama, Masashi

Search repository
アクセス権
アクセス権 open access
アクセス権URI http://purl.org/coar/access_right/c_abf2
権利
言語 en
権利情報 © 2017. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/
キーワード
主題Scheme Other
主題 acylceramide
キーワード
主題Scheme Other
主題 ceramide
キーワード
主題Scheme Other
主題 corneocyte lipid envelope
キーワード
主題Scheme Other
主題 EOS
キーワード
主題Scheme Other
主題 ichthyosis syndrome
抄録
内容記述 Research on the genetic abnormalities and pathogenetic processes of ichthyoses has progressed remarkably, and many causative genes and molecules have been identified in ichthyoses and ichthyosis syndromes. Most of the genes/molecules causative of ichthyosis are associated with the barrier function of the stratum corneum, and defects in the skin barrier play important roles in the pathogenesis of various ichthyosis phenotypes. It has been elucidated that, of the ichthyosis-causative genes, ABCA12, ALOXE3, ALOX12B, CYP4F22, CERS3, ABHD5, PNPLA1 and ELOVL4 work in the formation of the corneocyte lipid envelope (CLE), a structure that is essential to sound skin barrier function. The CLE mostly consists of ultra-long-chain (ULC) ceramides derived from ULC-acylceramide (EOS; a combination of esterified ω-hydroxy fatty acids and sphingosines). In this review, I shed light on the synthesis, metabolism and transport of epidermal ceramides, especially on ULC-acylceramide and the processes of CLE formation. In addition, I summarize the pathogeneses of various ichthyoses and ichthyosis syndromes from the aspects of abnormal synthesis of ULC-acylceramide and malformation of the CLE. Investigations on the pathomechanisms of ichthyoses have provided novel knowledge on the synthesis and metabolism of ceramides in the epidermis. Conversely, research on the dynamics of epidermal ceramides has contributed to the elucidation of the pathogenesis of ichthyoses. Advances in our understanding of the biology of epidermal lipids and the disease pathogeneses of ichthyoses and ichthyosis syndromes promise to provide clues for the development of effective therapies for ichthyosis patients in the near future.
言語 en
内容記述タイプ Abstract
出版者
言語 en
出版者 Elsevier
言語
言語 eng
資源タイプ
資源タイプresource http://purl.org/coar/resource_type/c_6501
タイプ journal article
出版タイプ
出版タイプ AM
出版タイプResource http://purl.org/coar/version/c_ab4af688f83e57aa
DOI
関連タイプ isVersionOf
識別子タイプ DOI
関連識別子 https://doi.org/10.1016/j.jdermsci.2017.06.002
ISSN
収録物識別子タイプ PISSN
収録物識別子 0923-1811
書誌情報 en : JOURNAL OF DERMATOLOGICAL SCIENCE

巻 88, p. 3-9, 発行日 2017-10
著者版フラグ
値 author
URI
識別子 http://doi.org/10.1016/j.jdermsci.2017.06.002
識別子タイプ DOI
URI
識別子 http://hdl.handle.net/2237/27314
識別子タイプ HDL
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