Detection of factor XIII inhibitors in 33 patients with autoimmune factor XIII deficiency in Japan.
Acquired bleeding disorder
Amine incorporation assay
Anti-factor XIII autoantibody
Factor XIII inhibitor
α2-plasmin inhibitor
Journal
International journal of hematology
ISSN: 1865-3774
Titre abrégé: Int J Hematol
Pays: Japan
ID NLM: 9111627
Informations de publication
Date de publication:
19 Jun 2024
19 Jun 2024
Historique:
received:
03
04
2024
accepted:
05
06
2024
revised:
31
05
2024
medline:
19
6
2024
pubmed:
19
6
2024
entrez:
19
6
2024
Statut:
aheadofprint
Résumé
Autoimmune factor XIII (FXIII) deficiency (AiF13D) is a rare hemorrhagic disease. The anti-FXIII autoantibodies that cause this disease are classified into three types: type Aa inhibits the heterotetramer assembly and activation of FXIII, type Ab inhibits the enzymatic activity of activated FXIII, and type B enhances the elimination of FXIII from the blood. The former two are FXIII inhibitors and may be lethal if overlooked by conventional functional assays. To reliably detect both types of FXIII inhibitors, a new assay was developed by incorporating 5-(biotinamido)pentylamine (BAPA) into α
Identifiants
pubmed: 38896335
doi: 10.1007/s12185-024-03807-y
pii: 10.1007/s12185-024-03807-y
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : the Japanese Ministry of Education, Culture, Sports, Science and Technology
ID : MEXT
Organisme : the Japanese Ministry of Education, Culture, Sports, Science and Technology
ID : 16K09820
Organisme : the Japan Agency for Medical Research and Development
ID : AMED
Organisme : the Japan Agency for Medical Research and Development
ID : 16ek0109043h0003
Organisme : the Japanese Ministry of Health, Labor, and Welfare
ID : MHLW
Organisme : the Japanese Ministry of Health, Labor, and Welfare
ID : 21FC1008
Informations de copyright
© 2024. Japanese Society of Hematology.
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