Inherited ADAMTS13 mutations associated with thrombotic thrombocytopenic purpura: a short review and update

Zoe Markham-Lee; Neil Morgan; Jonas Emsley

doi:10.1080/09537104.2022.2138306

Inherited ADAMTS13 mutations associated with thrombotic thrombocytopenic purpura: a short review and update

Zoe Markham-Lee^*, Neil Morgan^*, Jonas Emsley

^*Corresponding author for this work

Cardiovascular Sciences

Research output: Contribution to journal › Article › peer-review

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Abstract

ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein. ADAMTS13 is a multi-domain protein with the domains broadly functioning to interact and cleave VWF or maintain global latency of ADAMTS13. Thrombotic Thrombocytopenic Purpura is a disease characterized by excessive thrombi formation in the microvasculature, diagnosis is made when ADAMTS13 activity is

Original language	English
Journal	Platelets
Early online date	25 Oct 2022
DOIs	https://doi.org/10.1080/09537104.2022.2138306
Publication status	E-pub ahead of print - 25 Oct 2022

Keywords

ADAMTS13
Thrombotic Thrombocytopenic Purpura
VWF

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10.1080/09537104.2022.2138306Licence: Creative Commons: Attribution (CC BY)

MarkhamLeeZ2022InheritedFinal published version, 1.86 MBLicence: Creative Commons: Attribution (CC BY)

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abstract = "ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein. ADAMTS13 is a multi-domain protein with the domains broadly functioning to interact and cleave VWF or maintain global latency of ADAMTS13. Thrombotic Thrombocytopenic Purpura is a disease characterized by excessive thrombi formation in the microvasculature, diagnosis is made when ADAMTS13 activity is ",

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T1 - Inherited ADAMTS13 mutations associated with thrombotic thrombocytopenic purpura

T2 - a short review and update

AU - Markham-Lee, Zoe

AU - Morgan, Neil

AU - Emsley, Jonas

PY - 2022/10/25

Y1 - 2022/10/25

N2 - ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein. ADAMTS13 is a multi-domain protein with the domains broadly functioning to interact and cleave VWF or maintain global latency of ADAMTS13. Thrombotic Thrombocytopenic Purpura is a disease characterized by excessive thrombi formation in the microvasculature, diagnosis is made when ADAMTS13 activity is

AB - ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein. ADAMTS13 is a multi-domain protein with the domains broadly functioning to interact and cleave VWF or maintain global latency of ADAMTS13. Thrombotic Thrombocytopenic Purpura is a disease characterized by excessive thrombi formation in the microvasculature, diagnosis is made when ADAMTS13 activity is

KW - ADAMTS13

KW - Thrombotic Thrombocytopenic Purpura

KW - VWF

U2 - 10.1080/09537104.2022.2138306

DO - 10.1080/09537104.2022.2138306

M3 - Article

SN - 0953-7104

JO - Platelets

JF - Platelets

ER -

Inherited ADAMTS13 mutations associated with thrombotic thrombocytopenic purpura: a short review and update

Abstract

Keywords

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