VWF and ADAMTS13

Number of citations of the paper that reports this interaction (PubMedID 15824096)
0

Data Source:
HPRD (in vitro)

		VWF	ADAMTS13
Description		von Willebrand factor	ADAM metallopeptidase with thrombospondin type 1 motif 13
Image
GO Annotations	Cellular Component	Extracellular Region Extracellular Space Endoplasmic Reticulum Extracellular Matrix Platelet Alpha Granule Platelet Alpha Granule Lumen Weibel-Palade Body Extracellular Exosome Cell Periphery	Extracellular Region Extracellular Space Endoplasmic Reticulum Lumen Cell Surface Extracellular Matrix
	Molecular Function	Protease Binding Integrin Binding Extracellular Matrix Structural Constituent Protein Binding Collagen Binding Immunoglobulin Binding Identical Protein Binding Protein-folding Chaperone Binding	Endopeptidase Activity Metalloendopeptidase Activity Integrin Binding Calcium Ion Binding Protein Binding Peptidase Activity Metallopeptidase Activity Zinc Ion Binding Hydrolase Activity Metal Ion Binding
	Biological Process	Cell Adhesion Blood Coagulation Hemostasis Response To Wounding Platelet Activation Cell-substrate Adhesion Positive Regulation Of Intracellular Signal Transduction	Proteolysis Cell-matrix Adhesion Integrin-mediated Signaling Pathway Blood Coagulation Hemostasis Glycoprotein Metabolic Process Response To Toxic Substance Response To Amine Protein Processing Protein Catabolic Process Platelet Activation Extracellular Matrix Organization Response To Type II Interferon Response To Tumor Necrosis Factor Response To Potassium Ion Peptide Catabolic Process Response To Interleukin-4 Cellular Response To Lipopolysaccharide Cellular Response To Type II Interferon Cellular Response To Interleukin-4 Cellular Response To Tumor Necrosis Factor
Pathways		Platelet degranulation Intrinsic Pathway of Fibrin Clot Formation Integrin cell surface interactions Integrin signaling GRB2:SOS provides linkage to MAPK signaling for Integrins p130Cas linkage to MAPK signaling for integrins GP1b-IX-V activation signalling MAP2K and MAPK activation Signaling by moderate kinase activity BRAF mutants Signaling by high-kinase activity BRAF mutants Signaling by BRAF and RAF1 fusions Paradoxical activation of RAF signaling by kinase inactive BRAF Platelet Adhesion to exposed collagen Platelet Aggregation (Plug Formation) Signaling downstream of RAS mutants Signaling by RAF1 mutants Defective F8 cleavage by thrombin Defective F8 binding to von Willebrand factor Enhanced cleavage of VWF variant by ADAMTS13 Enhanced binding of GP1BA variant to VWF multimer:collagen Defective VWF cleavage by ADAMTS13 variant Defective VWF binding to collagen type I Defective binding of VWF variant to GPIb:IX:V	Defective B3GALTL causes PpS O-glycosylation of TSR domain-containing proteins Platelet Adhesion to exposed collagen Enhanced cleavage of VWF variant by ADAMTS13 Defective VWF cleavage by ADAMTS13 variant
Drugs		Antihemophilic factor, human recombinant Egaptivon pegol Caplacizumab Simoctocog alfa Antihemophilic factor (recombinant), PEGylated Susoctocog alfa Efmoroctocog alfa Lonoctocog alfa Moroctocog alfa Turoctocog alfa pegol Rurioctocog alfa pegol
Diseases		Hemophilia; Hemophilia A (HEMA); Hemophilia B (HEMB); von Willebrand disease (VWD)	Thrombotic thrombocytopenic purpura; Moschcowitz disease; Schulman-Upshaw syndrome
GWAS		Coagulation factor levels ( 23267103) Factor VIII levels ( 20231535 30586737) Low vWF levels ( 26486471) Mean platelet volume ( 22139419 27863252 32888494) Peginterferon alfa-2a treatment response in chronic hepatitis B infection ( 30972912) Platelet count ( 22139419 26805783 32888494) Response to tofacitinib treatment in psoriasis (herpes zoster) ( 33455090) Response to tofacitinib treatment in psoriasis (herpes zoster)(time to event) ( 33455090) Venous thromboembolism ( 31420334) vWF levels ( 20231535 30586737) vWF levels in ischaemic stroke and hyperhomocysteinaemia ( 28495826)	Activated partial thromboplastin time ( 22703881) ADAMTS13 activity ( 25934476) ADAMTS13 levels ( 29296746) Circulating fibroblast growth factor 23 levels ( 30217807) Coagulation factor levels ( 23267103) Cobalt levels ( 26025379) Gut microbiota (functional units) ( 27694959) Liver enzyme levels ( 18940312) Lymphocyte percentage of white cells ( 32888494) Malaria ( 31844061) Pancreatic cancer ( 25086665) vWF levels in ischaemic stroke and hyperhomocysteinaemia ( 28495826)
Interacting Genes		28 interacting genes: ADAMTS13 ALB APOA1 ATXN7 CACNA1A CALR COL1A1 COL1A2 COL3A1 F8 FGA FGB FGG FURIN FXR1 GP1BA HOXA1 HSPA5 ITGA2B LCOR MAP3K3 NUFIP2 P4HB PROS1 RAC1 SOCS4 TNFRSF11B ZNF512B	1 interacting genes: VWF
Entrez ID		7450	11093
HPRD ID		01906	04994
Ensembl ID		ENSG00000110799	ENSG00000160323
Uniprot IDs		P04275	Q76LX8
PDB IDs		1AO3 1ATZ 1AUQ 1FE8 1FNS 1IJB 1IJK 1M10 1OAK 1SQ0 1U0N 1UEX 2ADF 2MHP 2MHQ 3GXB 3HXO 3HXQ 3PPV 3PPW 3PPX 3PPY 3ZQK 4C29 4C2A 4C2B 4DMU 4NT5 5BV8 6FWN 6N29 7EOW 7F49 7KWO 7P4N 7PMV 7PNF 7ZWH 8D3C 8D3D	3GHM 3GHN 3VN4 6QIG 7B01
Enriched GO Terms of Interacting Partners?		Endoplasmic Reticulum Lumen Positive Regulation Of Substrate Adhesion-dependent Cell Spreading Regulation Of Substrate Adhesion-dependent Cell Spreading Fibrinolysis Protein Activation Cascade Cell-matrix Adhesion Coagulation Blood Coagulation Hemostasis Platelet Alpha Granule Lumen Blood Microparticle Platelet Activation Positive Regulation Of Cell-substrate Adhesion Protein Maturation Regulation Of Cell-substrate Adhesion Cell-substrate Adhesion Negative Regulation Of Blood Coagulation Negative Regulation Of Hemostasis Extracellular Space Negative Regulation Of Coagulation Extracellular Matrix Structural Constituent Positive Regulation Of Rho Protein Signal Transduction Regulation Of Blood Coagulation Negative Regulation Of Wound Healing Regulation Of Hemostasis Fibrinogen Complex Extracellular Region Extracellular Matrix Fibrillar Collagen Trimer Regulation Of Body Fluid Levels Blood Coagulation, Fibrin Clot Formation Zymogen Activation Protein Processing Regulation Of Biological Quality Platelet-derived Growth Factor Binding Plasminogen Activation Cell Surface Regulation Of Signal Transduction Regulation Of Signaling Protein Folding In Endoplasmic Reticulum Regulation Of Cell Communication Positive Regulation Of Heterotypic Cell-cell Adhesion External Side Of Plasma Membrane Negative Regulation Of Multicellular Organismal Process Extracellular Exosome Collagen Type I Trimer Extracellular Matrix Organization Regulation Of Wound Healing Extracellular Structure Organization Platelet Alpha Granule	Immunoglobulin Binding Weibel-Palade Body Set1C/COMPASS Complex NSL Complex Histone H3Q5ser Reader Activity MLL3/4 Complex Histone H3K4 Methyltransferase Activity ATAC Complex MLL1/2 Complex Histone H3K4me1 Reader Activity Cytoplasmic Exosome (RNase Complex) Exonucleolytic Trimming To Generate Mature 3'-end Of 5.8S RRNA From Tricistronic RRNA Transcript (SSU-rRNA, 5.8S RRNA, LSU-rRNA) 7S RNA Binding TRAMP-dependent TRNA Surveillance Pathway TRNA Surveillance TRNA Decay RRNA 3'-end Processing Nucleolar Exosome (RNase Complex) Nuclear Polyadenylation-dependent RRNA Catabolic Process RNA Exonuclease Activity U4 SnRNA 3'-end Processing CUT Catabolic Process Poly(A)-dependent SnoRNA 3'-end Processing Sno(s)RNA Metabolic Process Nuclear Exosome (RNase Complex) Exosome (RNase Complex) Platelet Alpha Granule SnRNA 3'-end Processing Positive Regulation Of Gluconeogenesis Histone Acetyltransferase Complex Histone Methyltransferase Complex 3'-5'-RNA Exonuclease Activity MLL1 Complex SnRNA Processing Nuclear RNA Surveillance RNA Surveillance Positive Regulation Of Glucose Metabolic Process Positive Regulation Of Gene Expression, Epigenetic Gluconeogenesis Regulation Of Gluconeogenesis Transcription Initiation-coupled Chromatin Remodeling SnRNA Metabolic Process Hexose Biosynthetic Process Platelet Alpha Granule Lumen Collagen Binding Platelet Activation
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