The Function of extravascular coagulation factor IX in haemostasis.


Journal

Haemophilia : the official journal of the World Federation of Hemophilia
ISSN: 1365-2516
Titre abrégé: Haemophilia
Pays: England
ID NLM: 9442916

Informations de publication

Date de publication:
May 2021
Historique:
revised: 07 03 2021
received: 09 02 2021
accepted: 09 03 2021
pubmed: 30 3 2021
medline: 25 9 2021
entrez: 29 3 2021
Statut: ppublish

Résumé

The majority of clotting factor IX (FIX) resides extravascularly, in the subendothelial basement membrane, where it is important for haemostasis. We summarize preclinical studies demonstrating extravascular FIX and its role in haemostasis and discuss clinical observations supporting this. We compare the in vivo binding of BeneFIX Three mouse models of haemophilia were used: the FIX knockout as the CRM CRM status affects recovery and prophylactic efficacy. Prophylactic protection decreases ~5X faster in CRM Preclinical and clinical results support the interpretation that FIX plays a role in haemostasis from its extravascular location. We believe that knowing the CRM status of haemophilia B patients is important for optimizing prophylactic dosing with less trial and error, thereby decreasing clinical morbidity.

Identifiants

pubmed: 33780107
doi: 10.1111/hae.14300
doi:

Substances chimiques

Factor IX 9001-28-9

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

332-339

Informations de copyright

© 2021 John Wiley & Sons Ltd.

Références

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Auteurs

David M Mann (DM)

Mann BioConsulting, Gaithersburg, MD, USA.

Katherine A Stafford (KA)

Department of Medicine, Mount Auburn Hospital, Cambridge, MA, USA.

Man-Chiu Poon (MC)

University of Calgary Foothills Medical Center, Calgary, AB, Canada.

Davide Matino (D)

McMaster University Medical Center, Hamilton, ON, Canada.

Darrel W Stafford (DW)

Department of Biology, UNC-Chapel Hill, Chapel Hill, NC, USA.

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