| 登録情報 | データベース: EMDB / ID: EMD-32620 |
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| タイトル | Cryo-EM structure of VWF D'D3 dimer (2M mutant) complexed with D1D2 at 3.29 angstron resolution (2 units) |
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マップデータ | |
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試料 | - 複合体: VWF D'D3-D1D2 complex (1 unit)
- タンパク質・ペプチド: von Willebrand antigen 2
- タンパク質・ペプチド: von Willebrand factor
- リガンド: CALCIUM ION
- リガンド: 2-acetamido-2-deoxy-beta-D-glucopyranose
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キーワード | blood / VWF / von Willebrand factor / von Willebrand disease / blood coagulation / blood clotting / multimer assembly / VWF assembly / D'D3 domain / D1D2 domain / D'D3 dimer / D1D2 Dimer / VWF Tube / repeating unit |
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| 機能・相同性 | 機能・相同性情報
Defective VWF binding to collagen type I / Enhanced cleavage of VWF variant by ADAMTS13 / Defective VWF cleavage by ADAMTS13 variant / Defective F8 binding to von Willebrand factor / Enhanced binding of GP1BA variant to VWF multimer:collagen / Defective binding of VWF variant to GPIb:IX:V / Weibel-Palade body / hemostasis / platelet alpha granule / Platelet Adhesion to exposed collagen ...Defective VWF binding to collagen type I / Enhanced cleavage of VWF variant by ADAMTS13 / Defective VWF cleavage by ADAMTS13 variant / Defective F8 binding to von Willebrand factor / Enhanced binding of GP1BA variant to VWF multimer:collagen / Defective binding of VWF variant to GPIb:IX:V / Weibel-Palade body / hemostasis / platelet alpha granule / Platelet Adhesion to exposed collagen / GP1b-IX-V activation signalling / extracellular matrix structural constituent / p130Cas linkage to MAPK signaling for integrins / Defective F8 cleavage by thrombin / Platelet Aggregation (Plug Formation) / cell-substrate adhesion / GRB2:SOS provides linkage to MAPK signaling for Integrins / positive regulation of intracellular signal transduction / immunoglobulin binding / Integrin cell surface interactions / collagen binding / : / Integrin signaling / platelet alpha granule lumen / Signaling by high-kinase activity BRAF mutants / MAP2K and MAPK activation / platelet activation / response to wounding / integrin binding / blood coagulation / Signaling by RAF1 mutants / Signaling by moderate kinase activity BRAF mutants / Paradoxical activation of RAF signaling by kinase inactive BRAF / Signaling downstream of RAS mutants / Signaling by BRAF and RAF1 fusions / Platelet degranulation / protein-folding chaperone binding / extracellular matrix / protease binding / cell adhesion / endoplasmic reticulum / : / extracellular exosome / extracellular region / identical protein binding類似検索 - 分子機能 von Willebrand factor, VWA N-terminal domain / Von Willebrand factor / VWA N-terminal / Von Willebrand factor-like domain / : / Otogelin-like/Mucin TIL domain / C8 domain / Uncharacterised domain, cysteine-rich / : / C8 ...von Willebrand factor, VWA N-terminal domain / Von Willebrand factor / VWA N-terminal / Von Willebrand factor-like domain / : / Otogelin-like/Mucin TIL domain / C8 domain / Uncharacterised domain, cysteine-rich / : / C8 / von Willebrand factor, type D domain / von Willebrand factor type D domain / VWFD domain profile. / von Willebrand factor (vWF) type D domain / Trypsin Inhibitor-like, cysteine rich domain / Serine protease inhibitor-like superfamily / Trypsin Inhibitor like cysteine rich domain / C-terminal cystine knot signature. / C-terminal cystine knot domain profile. / von Willebrand factor (vWF) type C domain / von Willebrand factor type C domain / Cystine knot, C-terminal / C-terminal cystine knot-like domain (CTCK) / VWFC domain signature. / VWFC domain profile. / von Willebrand factor (vWF) type C domain / VWFC domain / von Willebrand factor type A domain / VWFA domain profile. / von Willebrand factor (vWF) type A domain / von Willebrand factor, type A / von Willebrand factor A-like domain superfamily類似検索 - ドメイン・相同性 |
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| 生物種 | Homo sapiens (ヒト) |
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| 手法 | 単粒子再構成法 / クライオ電子顕微鏡法 / 解像度: 3.29 Å |
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データ登録者 | Zeng JW / Shu ZM / Zhou AW |
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| 資金援助 | 中国, 1件 | Organization | Grant number | 国 |
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| Ministry of Science and Technology (MoST, China) | 2016YFA0501103 | 中国 |
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引用 | ジャーナル: Cell Discov / 年: 2022 タイトル: Structural mechanism of VWF D'D3 dimer formation. 著者: Zimei Shu / Jianwei Zeng / Li Xia / Haiyan Cai / Aiwu Zhou /  |
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| 履歴 | | 登録 | 2022年1月17日 | - |
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| ヘッダ(付随情報) 公開 | 2022年5月25日 | - |
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| マップ公開 | 2022年5月25日 | - |
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| 更新 | 2024年10月16日 | - |
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| 現状 | 2024年10月16日 | 処理サイト: PDBj / 状態: 公開 |
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