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Molecular basis of sugar recognition by collectin-K1 and the effects of mutations associated with 3MC syndrome

https://asahikawa-med.repo.nii.ac.jp/records/5517
https://asahikawa-med.repo.nii.ac.jp/records/5517
23dbb21c-1659-4b84-b626-97cf47634c02
名前 / ファイル ライセンス アクション
6528.pdf 6528.pdf (3.2 MB)
Item type 学術雑誌論文 / Journal Article_02(1)
公開日 2016-08-17
タイトル
タイトル Molecular basis of sugar recognition by collectin-K1 and the effects of mutations associated with 3MC syndrome
言語
言語 eng
キーワード
主題Scheme Other
キーワード Complement activation
キーワード
主題Scheme Other
キーワード structural biology
キーワード
主題Scheme Other
キーワード C-type lectin
キーワード
主題Scheme Other
キーワード 3MC syndrome
資源タイプ
資源タイプ journal article
著者 Umakhanth, Venkatraman Girija

× Umakhanth, Venkatraman Girija

Umakhanth, Venkatraman Girija

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Christopher, M Furze

× Christopher, M Furze

Christopher, M Furze

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Alexandre, R Gingras

× Alexandre, R Gingras

Alexandre, R Gingras

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吉崎, 隆之

× 吉崎, 隆之

吉崎, 隆之

ja-Kana ヨシザキ, タカユキ

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大谷, 克城

× 大谷, 克城

大谷, 克城

ja-Kana オオタニ, カツキ

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Jamie, E Marshall

× Jamie, E Marshall

Jamie, E Marshall

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A, Katrine Wallis

× A, Katrine Wallis

A, Katrine Wallis

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Wilhelm, J Schwaeble

× Wilhelm, J Schwaeble

Wilhelm, J Schwaeble

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Mohammed, El-Mezgueldi

× Mohammed, El-Mezgueldi

Mohammed, El-Mezgueldi

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Daniel, A Mitchell

× Daniel, A Mitchell

Daniel, A Mitchell

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Peter, CE Moody

× Peter, CE Moody

Peter, CE Moody

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若宮, 伸隆

× 若宮, 伸隆

若宮, 伸隆

ja-Kana ワカミヤ, ノブタカ

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Russell, Wallis

× Russell, Wallis

Russell, Wallis

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著者 ローマ字
著者 ローマ字
著者 ローマ字
著者 ローマ字
Yoshizaki, Takayuki
著者 ローマ字
Ohtani, Katsuki
著者 ローマ字
著者 ローマ字
著者 ローマ字
著者 ローマ字
著者 ローマ字
著者 ローマ字
著者 ローマ字
Wakamiya, Nobutaka
書誌情報 BMC biology

巻 13, 号 27, p. 1-14, 発行日 2015-01-01
ISSN
収録物識別子タイプ ISSN
収録物識別子 1741-7007
DOI
関連タイプ isIdenticalTo
識別子タイプ DOI
関連識別子 10.1186/s12915-015-0136-2
リンクURL
内容記述タイプ Other
内容記述 http://bmcbiol.biomedcentral.com/articles/10.1186/s12915-015-0136-2 | http://bmcbiol.biomedcentral.com/articles/10.1186/s12915-015-0136-2
識別番号 その他
内容記述タイプ Other
内容記述 PMID:25912189
抄録
内容記述タイプ Abstract
内容記述 BACKGROUND:
\nCollectin-K1 (CL-K1, or CL-11) is a multifunctional Ca(2+)-dependent lectin with roles in innate immunity, apoptosis and embryogenesis. It binds to carbohydrates on pathogens to activate the lectin pathway of complement and together with its associated serine protease MASP-3 serves as a guidance cue for neural crest development. High serum levels are associated with disseminated intravascular coagulation, where spontaneous clotting can lead to multiple organ failure. Autosomal mutations in the CL-K1 or MASP-3 genes cause a developmental disorder called 3MC (Carnevale, Mingarelli, Malpuech and Michels) syndrome, characterised by facial, genital, renal and limb abnormalities. One of these mutations (Gly(204)Ser in the CL-K1 gene) is associated with undetectable levels of protein in the serum of affected individuals.
RESULTS:
\nIn this study, we show that CL-K1 primarily targets a subset of high-mannose oligosaccharides present on both self- and non-self structures, and provide the structural basis for its ligand specificity. We also demonstrate that three disease-associated mutations prevent secretion of CL-K1 from mammalian cells, accounting for the protein deficiency observed in patients. Interestingly, none of the mutations prevent folding or oligomerization of recombinant fragments containing the mutations in vitro. Instead, they prevent Ca(2+) binding by the carbohydrate-recognition domains of CL-K1. We propose that failure to bind Ca(2+) during biosynthesis leads to structural defects that prevent secretion of CL-K1, thus providing a molecular explanation of the genetic disorder.
CONCLUSIONS:
\nWe have established the sugar specificity of CL-K1 and demonstrated that it targets high-mannose oligosaccharides on self- and non-self structures via an extended binding site which recognises the terminal two mannose residues of the carbohydrate ligand. We have also shown that mutations associated with a rare developmental disorder called 3MC syndrome prevent the secretion of CL-K1, probably as a result of structural defects caused by disruption of Ca(2+) binding during biosynthesis
注記
内容記述タイプ Other
注記 Creative Commons Attribution License4.0
資源タイプ
内容記述タイプ Other
資源タイプ text
著者版フラグ
出版タイプ VoR
フォーマット
内容記述タイプ Other
内容記述 application/pdf
ID(XooNIps)
25912189
閲覧数(XooNIps)
ダウンロード数(XooNIps)
897
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Cite as

Umakhanth, Venkatraman Girija, Christopher, M Furze, Alexandre, R Gingras, 吉崎, 隆之, 大谷, 克城, Jamie, E Marshall, A, Katrine Wallis, Wilhelm, J Schwaeble, Mohammed, El-Mezgueldi, Daniel, A Mitchell, Peter, CE Moody, 若宮, 伸隆, Russell, Wallis, n.d., Molecular basis of sugar recognition by collectin-K1 and the effects of mutations associated with 3MC syndrome: 1–14 p.

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