Preferred Label : ADAMTS13 Protein;

MeSH definition : An ADAMTS protease that contains eight thrombospondin (TS) motifs. It cleaves VON WILLEBRAND FACTOR to control vWF-mediated THROMBOSIS. Mutations in the ADAMTS13 gene have been identified in familial cases of PURPURA, THROMBOTIC THROMBOCYTOPENIC and defects in ADAMTS13 activity are associated with MYOCARDIAL INFARCTION; BRAIN ISCHEMIA; PRE-ECLAMPSIA; and MALARIA.;

MeSH synonym : von Willebrand Factor-Cleaving Protease; von Willebrand Factor Cleaving Protease; ADAMTS13 Protease; vWF-Cleaving Protease; vWF Cleaving Protease; ADAMTS-13 Protein; ADAMTS 13 Protein; A Disintegrin and Metalloproteinase with Thrombospondin Motifs 13 Protein;

Registry Number MeSH : EC 3.4.24.87;

UNII : EC 3.4.24.87;

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An ADAMTS protease that contains eight thrombospondin (TS) motifs. It cleaves VON WILLEBRAND FACTOR to control vWF-mediated THROMBOSIS. Mutations in the ADAMTS13 gene have been identified in familial cases of PURPURA, THROMBOTIC THROMBOCYTOPENIC and defects in ADAMTS13 activity are associated with MYOCARDIAL INFARCTION; BRAIN ISCHEMIA; PRE-ECLAMPSIA; and MALARIA.

https://www.has-sante.fr/jcms/p_3586231/fr/adzynma-adamts13r-purpura-thrombotique-thrombocytopenique-congenital-pttc
2025
false
false
false
France
ADAMTS13 Protein
Apadamtase Alfa
enzyme replacement therapy
congenital thrombotic thrombocytopenic purpura
adult
child
treatment outcome
insurance, health, reimbursement
orphan drug production
injections, intravenous
Congenital thrombotic thrombocytopenic purpura
evaluation of the transparency committee
purpura, thrombotic thrombocytopenic

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https://ansm.sante.fr/tableau-marr/adamts13r
2024
false
false
false
France
French
risk management
ADAMTS13 Protein
guidelines for drug use
drug hypersensitivity
drug hypersensitivity
signs and symptoms
patient education handout
enzyme replacement therapy
adult
child
congenital thrombotic thrombocytopenic purpura
ADAMTS13 Protein
recombinant proteins

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https://www.ema.europa.eu/en/medicines/human/EPAR/adzynma
2024
false
false
false
Netherlands
French
English
treatment outcome
syndication feed
summary of product characteristics
package leaflet
drug evaluation
orphan drug production
child
adult
congenital thrombotic thrombocytopenic purpura
enzyme replacement therapy
ADAMTS13 protein, human
recombinant proteins
ADAMTS13 Protein
ADAMTS13 Protein
risk management
pregnancy
breast feeding
drug approval
europe
injections, intravenous
von willebrand factor
product surveillance, postmarketing
aged
apadamtase alfa and cinaxadamtase alfa

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03/05/2025


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