| 登録情報 | データベース: PDB / ID: 9jbv |
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| タイトル | Structure of Engineered Coagulation Factor VIII with Enhanced Secretion and Coagulation Potential |
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要素 | Coagulation factor VIII |
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キーワード | BLOOD CLOTTING / hemophilia |
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| 機能・相同性 | 機能・相同性情報
Defective F8 accelerates dissociation of the A2 domain / Defective F8 binding to the cell membrane / Defective F8 secretion / Defective F8 sulfation at Y1699 / Gamma carboxylation, hypusinylation, hydroxylation, and arylsulfatase activation / Defective F8 binding to von Willebrand factor / blood coagulation, intrinsic pathway / Cargo concentration in the ER / Defective factor IX causes thrombophilia / Defective cofactor function of FVIIIa variant ...Defective F8 accelerates dissociation of the A2 domain / Defective F8 binding to the cell membrane / Defective F8 secretion / Defective F8 sulfation at Y1699 / Gamma carboxylation, hypusinylation, hydroxylation, and arylsulfatase activation / Defective F8 binding to von Willebrand factor / blood coagulation, intrinsic pathway / Cargo concentration in the ER / Defective factor IX causes thrombophilia / Defective cofactor function of FVIIIa variant / Defective F9 variant does not activate FX / COPII-coated ER to Golgi transport vesicle / COPII-mediated vesicle transport / Defective F8 cleavage by thrombin / Common Pathway of Fibrin Clot Formation / Intrinsic Pathway of Fibrin Clot Formation / endoplasmic reticulum-Golgi intermediate compartment membrane / platelet alpha granule lumen / acute-phase response / Golgi lumen / blood coagulation / Platelet degranulation / oxidoreductase activity / endoplasmic reticulum lumen / copper ion binding / extracellular space / extracellular region / plasma membrane類似検索 - 分子機能 Coagulation factor 5/8-like / : / Coagulation factors 5/8 type C domain (FA58C) signature 2. / Multicopper oxidases, conserved site / Multicopper oxidases signature 1. / Coagulation factors 5/8 type C domain (FA58C) signature 1. / Coagulation factor 5/8 C-terminal domain, discoidin domain / Coagulation factors 5/8 type C domain (FA58C) profile. / Multicopper oxidase, C-terminal / Multicopper oxidase ...Coagulation factor 5/8-like / : / Coagulation factors 5/8 type C domain (FA58C) signature 2. / Multicopper oxidases, conserved site / Multicopper oxidases signature 1. / Coagulation factors 5/8 type C domain (FA58C) signature 1. / Coagulation factor 5/8 C-terminal domain, discoidin domain / Coagulation factors 5/8 type C domain (FA58C) profile. / Multicopper oxidase, C-terminal / Multicopper oxidase / F5/8 type C domain / Coagulation factor 5/8 C-terminal domain / Multicopper oxidase, N-terminal / Multicopper oxidase / Cupredoxin / Galactose-binding-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 | 電子顕微鏡法 / 単粒子再構成法 / クライオ電子顕微鏡法 / 解像度: 3.17 Å |
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データ登録者 | Kio, H. / Osamu, N. / Tsukasa, O. |
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| 資金援助 | 日本, 1件 | 組織 | 認可番号 | 国 |
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| Japan Agency for Medical Research and Development (AMED) | | 日本 |
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引用 | ジャーナル: To Be Published タイトル: Engineered Coagulation Factor VIII with Enhanced Secretion and Coagulation Potential for Hemophilia Gene Therapy 著者: Yuji, K. / Yuto, N. / Kio, H. / Tiago, L. / Yuma, F. / Yuki, Y. / Nemekhbayar, B. / Morisada, H. / Yuko, K. / Susumu, U. / Osamu, N. / Keiji, N. / Tsukasa, O. |
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| 履歴 | | 登録 | 2024年8月27日 | 登録サイト: PDBJ / 処理サイト: PDBJ |
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| 改定 1.0 | 2025年9月10日 | Provider: repository / タイプ: Initial release |
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| 改定 1.0 | 2025年9月10日 | Data content type: EM metadata / Data content type: EM metadata / Provider: repository / タイプ: Initial release |
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| 改定 1.0 | 2025年9月10日 | Data content type: FSC / Data content type: FSC / Provider: repository / タイプ: Initial release |
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| 改定 1.0 | 2025年9月10日 | Data content type: Half map / Part number: 1 / Data content type: Half map / Provider: repository / タイプ: Initial release |
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| 改定 1.0 | 2025年9月10日 | Data content type: Half map / Part number: 2 / Data content type: Half map / Provider: repository / タイプ: Initial release |
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| 改定 1.0 | 2025年9月10日 | Data content type: Image / Data content type: Image / Provider: repository / タイプ: Initial release |
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| 改定 1.0 | 2025年9月10日 | Data content type: Mask / Part number: 1 / Data content type: Mask / Provider: repository / タイプ: Initial release |
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| 改定 1.0 | 2025年9月10日 | Data content type: Primary map / Data content type: Primary map / Provider: repository / タイプ: Initial release |
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