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Guidelines

MIRAGE

ADAM metallopeptidase with thrombospondin type 1 motif 13

Summary

Gene Symbol

ADAMTS13

Organism

Homo sapiens (human)

External Links

NCBI Gene

11093

PubChem

11093

Alliance of Genome Resources

HGNC:1366

Annotation

Keyword

3D-structure
Alternative splicing
Blood coagulation
Calcium
Cleavage on pair of basic residues
Direct protein sequencing
Disease variant
Disulfide bond
Glycoprotein
Metal-binding
Metalloprotease
Proteomics identification
Reference proteome
Repeat
Secreted
Signal
Zinc
Zymogen

Proteins

Displaying 1 entry
UniProt	Protein Name
Q76LX8	von Willebrand factor-cleaving protease

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 18 in total

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GO Term	Evidence Code	PMID
proteolysis	IDA	11535495
proteolysis	IBA
cell-matrix adhesion	NAS	11557746
integrin-mediated signaling pathway	NAS	11557746
blood coagulation	TAS

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

amide metabolic process [inference]

peptide metabolic process [inference]

peptide catabolic process

organonitrogen compound metabolic process [inference]

peptide metabolic process [inference]

peptide catabolic process

protein metabolic process [inference]

protein processing

protein maturation [inference]

protein processing

organonitrogen compound catabolic process [inference]

catabolic process [inference]

cellular catabolic process [inference]

peptide catabolic process

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process

organonitrogen compound catabolic process [inference]

cellular metabolic process [inference]

cellular catabolic process [inference]

peptide catabolic process

primary metabolic process [inference]

protein metabolic process [inference]

protein processing

protein maturation [inference]

protein processing

organic substance metabolic process [inference]

macromolecule metabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process

protein metabolic process [inference]

protein processing

protein maturation [inference]

protein processing

carbohydrate derivative metabolic process [inference]

glycoprotein metabolic process

organonitrogen compound metabolic process [inference]

peptide metabolic process [inference]

peptide catabolic process

protein metabolic process [inference]

protein processing

protein maturation [inference]

protein processing

organonitrogen compound catabolic process [inference]

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process

organonitrogen compound catabolic process [inference]

cellular process [inference]

cell activation [inference]

platelet activation

cell adhesion [inference]

cell-substrate adhesion [inference]

cell-matrix adhesion

signal transduction [inference]

cell surface receptor signaling pathway [inference]

integrin-mediated signaling pathway

cellular metabolic process [inference]

cellular catabolic process [inference]

peptide catabolic process

cellular response to stimulus [inference]

cellular response to chemical stimulus [inference]

cellular response to organic substance [inference]

cellular response to cytokine stimulus [inference]

cellular response to lipid [inference]

cellular response to lipopolysaccharide

cellular response to oxygen-containing compound [inference]

cellular response to lipopolysaccharide

cellular response to biotic stimulus [inference]

cellular response to molecule of bacterial origin [inference]

cellular response to lipopolysaccharide

cellular component organization or biogenesis [inference]

cellular component organization [inference]

extracellular structure organization [inference]

extracellular matrix organization

external encapsulating structure organization [inference]

extracellular matrix organization

multicellular organismal process [inference]

cell activation [inference]

platelet activation

coagulation [inference]

blood coagulation

response to stimulus [inference]

response to external stimulus [inference]

response to external biotic stimulus [inference]

response to molecule of bacterial origin [inference]

response to lipopolysaccharide [inference]

cellular response to lipopolysaccharide

cellular response to molecule of bacterial origin [inference]

cellular response to lipopolysaccharide

response to biotic stimulus [inference]

response to external biotic stimulus [inference]

response to molecule of bacterial origin [inference]

response to lipopolysaccharide [inference]

cellular response to lipopolysaccharide

cellular response to molecule of bacterial origin [inference]

cellular response to lipopolysaccharide

cellular response to biotic stimulus [inference]

cellular response to molecule of bacterial origin [inference]

cellular response to lipopolysaccharide

response to chemical [inference]

response to toxic substance

response to organic substance [inference]

response to molecule of bacterial origin [inference]

response to lipopolysaccharide [inference]

cellular response to lipopolysaccharide

cellular response to molecule of bacterial origin [inference]

cellular response to lipopolysaccharide

response to organonitrogen compound [inference]

response to amine

response to lipid [inference]

response to lipopolysaccharide [inference]

cellular response to lipopolysaccharide

cellular response to lipid [inference]

cellular response to lipopolysaccharide

response to cytokine [inference]

response to type II interferon [inference]

cellular response to type II interferon

response to tumor necrosis factor [inference]

cellular response to tumor necrosis factor

response to interleukin-4 [inference]

cellular response to interleukin-4

cellular response to cytokine stimulus [inference]

cellular response to organic substance [inference]

cellular response to cytokine stimulus [inference]

cellular response to lipid [inference]

cellular response to lipopolysaccharide

response to inorganic substance [inference]

response to metal ion [inference]

response to potassium ion

cellular response to chemical stimulus [inference]

cellular response to organic substance [inference]

cellular response to cytokine stimulus [inference]

cellular response to lipid [inference]

cellular response to lipopolysaccharide

cellular response to oxygen-containing compound [inference]

cellular response to lipopolysaccharide

response to nitrogen compound [inference]

response to organonitrogen compound [inference]

response to amine

response to oxygen-containing compound [inference]

response to lipopolysaccharide [inference]

cellular response to lipopolysaccharide

response to potassium ion

cellular response to oxygen-containing compound [inference]

cellular response to lipopolysaccharide

response to salt [inference]

response to potassium ion

cellular response to stimulus [inference]

cellular response to chemical stimulus [inference]

cellular response to organic substance [inference]

cellular response to cytokine stimulus [inference]

cellular response to lipid [inference]

cellular response to lipopolysaccharide

cellular response to oxygen-containing compound [inference]

cellular response to lipopolysaccharide

cellular response to biotic stimulus [inference]

cellular response to molecule of bacterial origin [inference]

cellular response to lipopolysaccharide

biological regulation [inference]

regulation of biological process [inference]

regulation of cellular process [inference]

signal transduction [inference]

cell surface receptor signaling pathway [inference]

integrin-mediated signaling pathway

regulation of biological quality [inference]

regulation of body fluid levels [inference]

hemostasis [inference]

blood coagulation

Displaying entries **1 - 5** of 6 in total

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GO Term	Evidence Code	PMID
extracellular region	TAS
extracellular space	IEA
endoplasmic reticulum lumen	TAS
cell surface	NAS	11557746
extracellular matrix	TAS	11557746

GO Hierarchy

cellular component

cellular anatomical entity [inference]

external encapsulating structure [inference]

extracellular matrix

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

endoplasmic reticulum lumen

Displaying entries **1 - 5** of 8 in total

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GO Term	Evidence Code	PMID
metalloendopeptidase activity	EXP	12775718 18983500 31439947
metalloendopeptidase activity	TAS
metalloendopeptidase activity	IBA
integrin binding	TAS	11557746
calcium ion binding	TAS	11557746

GO Hierarchy

molecular function

catalytic activity [inference]

hydrolase activity [inference]

peptidase activity [inference]

endopeptidase activity [inference]

metalloendopeptidase activity

metallopeptidase activity

metalloendopeptidase activity

catalytic activity, acting on a protein [inference]

peptidase activity [inference]

endopeptidase activity [inference]

metalloendopeptidase activity

metallopeptidase activity

metalloendopeptidase activity

binding [inference]

protein binding

signaling receptor binding [inference]

integrin binding

cell adhesion molecule binding [inference]

integrin binding

ion binding [inference]

cation binding [inference]

metal ion binding [inference]

calcium ion binding

transition metal ion binding [inference]

zinc ion binding

protein-containing complex binding [inference]

integrin binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

a disintegrin and metalloproteinase with thrombospondin motifs 13

Functional Category

G: Carbohydrate transport and metabolism
T: Signal transduction mechanisms
X: Mobilome: prophages, transposons

Displaying **all 3** entries
Gene Ontology
extracellular matrix organization
metalloendopeptidase activity
proteolysis

Displaying **all 4** entries
InterPro
ADAMTS/ADAMTS-like, Spacer 1
ADAMTS/ADAMTS-like, cysteine-rich domain 3
ADAMTS/ADAMTS-like
Thrombospondin type-1 (TSP1) repeat

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:10772	thrombotic thrombocytopenic purpura	Alliance of Genome Resources

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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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Last updated: December 8, 2025