登録情報 データベース : PDB / ID : 1sq0 構造の表示 ダウンロードとリンクタイトル Crystal Structure of the Complex of the Wild-type Von Willebrand Factor A1 domain and Glycoprotein Ib alpha at 2.6 Angstrom Resolution 要素Platelet glycoprotein Ib alpha chain (Glycoprotein Ibalpha) (GP-Ib alpha) (GPIbA) (GPIb-alpha) (CD42B-alpha) (CD42B) [Contains: Glycocalicin] Von Willebrand factor (vWF) [Contains: Von Willebrand antigen II] 詳細キーワード BLOOD CLOTTING / Leucine Rich Repeat (LRR) / right-handed beta-alpha superhelix) / Integrin A (or I) domain fold機能・相同性 機能・相同性情報分子機能 ドメイン・相同性 構成要素
thrombin-activated receptor activity / glycoprotein Ib-IX-V complex / Defective VWF binding to collagen type I / Enhanced cleavage of VWF variant by ADAMTS13 / Defective VWF cleavage by ADAMTS13 variant / Weibel-Palade body / 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 / positive regulation of leukocyte tethering or rolling ... thrombin-activated receptor activity / glycoprotein Ib-IX-V complex / Defective VWF binding to collagen type I / Enhanced cleavage of VWF variant by ADAMTS13 / Defective VWF cleavage by ADAMTS13 variant / Weibel-Palade body / 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 / positive regulation of leukocyte tethering or rolling / hemostasis / blood coagulation, intrinsic pathway / Defective F9 activation / platelet alpha granule / Platelet Adhesion to exposed collagen / positive regulation of platelet activation / positive regulation of intracellular signal transduction / megakaryocyte development / GP1b-IX-V activation signalling / p130Cas linkage to MAPK signaling for integrins / cell-substrate adhesion / regulation of blood coagulation / Defective F8 cleavage by thrombin / Platelet Aggregation (Plug Formation) / immunoglobulin binding / GRB2:SOS provides linkage to MAPK signaling for Integrins / Integrin cell surface interactions / release of sequestered calcium ion into cytosol / fibrinolysis / collagen binding / Intrinsic Pathway of Fibrin Clot Formation / Integrin signaling / extracellular matrix / platelet alpha granule lumen / Signaling by high-kinase activity BRAF mutants / RUNX1 regulates genes involved in megakaryocyte differentiation and platelet function / MAP2K and MAPK activation / cell morphogenesis / platelet activation / response to wounding / 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 / blood coagulation / integrin binding / Platelet degranulation / protein-folding chaperone binding / collagen-containing extracellular matrix / protease binding / cell surface receptor signaling pathway / cell adhesion / external side of plasma membrane / cell surface / endoplasmic reticulum / extracellular space / extracellular exosome / extracellular region / identical protein binding / membrane / plasma membrane 類似検索 - 分子機能 von Willebrand factor, VWA N-terminal domain / Von Willebrand factor / VWA N-terminal / 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 ... von Willebrand factor, VWA N-terminal domain / Von Willebrand factor / VWA N-terminal / 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 / C-terminal cystine knot signature. / von Willebrand factor (vWF) type C domain / Trypsin Inhibitor-like, cysteine rich domain / Serine protease inhibitor-like superfamily / Trypsin Inhibitor like cysteine rich domain / C-terminal cystine knot domain profile. / Leucine rich repeat N-terminal domain / Cystine knot, C-terminal / C-terminal cystine knot-like domain (CTCK) / von Willebrand factor type C domain / Leucine-rich repeat N-terminal domain / Leucine rich repeat N-terminal domain / VWFC domain signature. / VWFC domain profile. / von Willebrand factor (vWF) type C domain / VWFC domain / von Willebrand factor, type A domain / Leucine-rich repeats, bacterial type / Cysteine-rich flanking region, C-terminal / Leucine rich repeat C-terminal domain / Leucine-rich repeat, LRR (right-handed beta-alpha superhelix) / Ribonuclease Inhibitor / von Willebrand factor type A domain / Alpha-Beta Horseshoe / von Willebrand factor (vWF) type A domain / VWFA domain profile. / Leucine rich repeat / Leucine-rich repeat, typical subtype / Leucine-rich repeats, typical (most populated) subfamily / von Willebrand factor, type A / Leucine-rich repeat profile. / von Willebrand factor A-like domain superfamily / Leucine-rich repeat / Leucine-rich repeat domain superfamily / Rossmann fold / 3-Layer(aba) Sandwich / Alpha Beta 類似検索 - ドメイン・相同性 von Willebrand factor / Platelet glycoprotein Ib alpha chain 類似検索 - 構成要素生物種 Homo sapiens (ヒト)手法 X線回折 / シンクロトロン / 多波長異常分散 / 解像度 : 2.6 Å 詳細データ登録者 Dumas, J.J. / Kumar, R. / McDonagh, T. / Sullivan, F. / Stahl, M.L. / Somers, W.S. / Mosyak, L. 引用 残り1件を表示 表示を減らす履歴 登録 2004年3月17日 登録サイト : RCSB / 処理サイト : RCSB改定 1.0 2004年4月13日 Provider : repository / タイプ : Initial release改定 1.1 2008年4月29日 Group : Version format compliance改定 1.2 2011年7月13日 Group : Version format compliance改定 1.3 2021年10月27日 Group : Database references / カテゴリ : database_2 / struct_ref_seq_difItem : _database_2.pdbx_DOI / _database_2.pdbx_database_accession / _struct_ref_seq_dif.details
すべて表示 表示を減らす Remark 999 SEQUENCE ASN RESIDUES AT POSITIONS 21 AND 159 OF CHAIN B, WHICH ARE SITES FOR N-LINKED ... SEQUENCE ASN RESIDUES AT POSITIONS 21 AND 159 OF CHAIN B, WHICH ARE SITES FOR N-LINKED GLYCOSYLATION, WERE MUTATED TO ASP.