登録情報 | データベース: PDB / ID: 7k66 |
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タイトル | Structure of Blood Coagulation Factor VIII in Complex with an Anti-C1 Domain Pathogenic Antibody Inhibitor |
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要素 | - 2A9 heavy chain
- 2A9 light chain
- Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII
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キーワード | BLOOD CLOTTING/Immune System / Antibody / inhibitor / BLOOD CLOTTING / BLOOD CLOTTING-Immune System complex |
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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 / Gamma carboxylation, hypusinylation, hydroxylation, and arylsulfatase activation / Defective F8 sulfation at Y1699 / 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 / Gamma carboxylation, hypusinylation, hydroxylation, and arylsulfatase activation / Defective F8 sulfation at Y1699 / 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-mediated vesicle transport / COPII-coated ER to Golgi transport vesicle / 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 / signaling receptor activity / oxidoreductase activity / copper ion binding / endoplasmic reticulum lumen / 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 / F5/8 type C domain ...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, type 1 / Multicopper oxidase / Multicopper oxidase, N-terminal / Multicopper oxidase / Cupredoxin / Galactose-binding-like domain superfamily類似検索 - ドメイン・相同性 COPPER (II) ION / Coagulation factor VIII / Coagulation factor VIII類似検索 - 構成要素 |
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生物種 | ![](img/tx_mammal.gif) Sus scrofa (ブタ)
Homo sapiens (ヒト)
![](img/tx_mammal.gif) Mus musculus (ハツカネズミ) |
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手法 | X線回折 / シンクロトロン / 分子置換 / 解像度: 3.92 Å |
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データ登録者 | Childers, K.C. / Gish, J. / Jarvis, L. / Peters, S. / Garrels, C. / Smith, I.W. / Spencer, H.T. / Spiegel, P.C. |
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資金援助 | 米国, 6件 組織 | 認可番号 | 国 |
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National Science Foundation (NSF, United States) | MRI 1429164 | 米国 | National Institutes of Health/National Heart, Lung, and Blood Institute (NIH/NHLBI) | R15HL103518 | 米国 | National Institutes of Health/National Heart, Lung, and Blood Institute (NIH/NHLBI) | U54HL141981 | 米国 | National Institutes of Health/National Heart, Lung, and Blood Institute (NIH/NHLBI) | R44HL117511 | 米国 | National Institutes of Health/National Heart, Lung, and Blood Institute (NIH/NHLBI) | R44HL110448 | 米国 | National Institutes of Health/National Heart, Lung, and Blood Institute (NIH/NHLBI) | U54HL112309 | 米国 |
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引用 | ジャーナル: Blood / 年: 2021 タイトル: Structure of blood coagulation factor VIII in complex with an anti-C1 domain pathogenic antibody inhibitor. 著者: Gish, J.S. / Jarvis, L. / Childers, K.C. / Peters, S.C. / Garrels, C.S. / Smith, I.W. / Spencer, H.T. / Doering, C.B. / Lollar, P. / Spiegel, P.C. |
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履歴 | 登録 | 2020年9月18日 | 登録サイト: RCSB / 処理サイト: RCSB |
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改定 1.0 | 2020年10月14日 | Provider: repository / タイプ: Initial release |
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改定 1.1 | 2021年2月24日 | Group: Database references / カテゴリ: citation / citation_author Item: _citation.country / _citation.journal_abbrev ..._citation.country / _citation.journal_abbrev / _citation.journal_id_CSD / _citation.journal_id_ISSN / _citation.pdbx_database_id_DOI / _citation.pdbx_database_id_PubMed / _citation.title / _citation.year / _citation_author.identifier_ORCID / _citation_author.name |
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改定 1.2 | 2021年6月9日 | Group: Database references / カテゴリ: citation / citation_author Item: _citation.journal_volume / _citation.page_first ..._citation.journal_volume / _citation.page_first / _citation.page_last / _citation.title / _citation_author.name |
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改定 1.3 | 2023年10月18日 | Group: Data collection / Database references / Refinement description カテゴリ: chem_comp_atom / chem_comp_bond ...chem_comp_atom / chem_comp_bond / database_2 / pdbx_initial_refinement_model Item: _database_2.pdbx_DOI / _database_2.pdbx_database_accession |
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