| 登録情報 | データベース: EMDB / ID: EMD-61327 |
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| タイトル | Structure of Engineered Coagulation Factor VIII with Enhanced Secretion and Coagulation Potential |
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マップデータ | unsharpened map |
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試料 | - 複合体: Engineered Coagulation Factor VIII with Enhanced Secretion and Coagulation Potential
- タンパク質・ペプチド: Coagulation factor VIII
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キーワード | hemophilia / BLOOD CLOTTING |
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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 | 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 | Organization | Grant number | 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 | - |
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| ヘッダ(付随情報) 公開 | 2025年9月10日 | - |
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| マップ公開 | 2025年9月10日 | - |
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| 更新 | 2025年9月10日 | - |
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| 現状 | 2025年9月10日 | 処理サイト: PDBj / 状態: 公開 |
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