Results 51 to 60 of about 12,566,431 (163)

A Sequential Mechanism for Exosite-mediated Factor IX Activation by Factor XIa*

Journal of Biological Chemistry, 2012
Background: Factor XIa proteolytically activates factor IX. Results: XIa cleaves IX after Arg145, forming IXα, and then after Arg180, forming IXaβ. Both reactions require substrate binding to the XIa A3 domain.
Yipeng Geng, I. Verhamme, A. Messer, Mao-fu Sun, Stephen B. Smith, S. Bajaj, D. Gailani   +6 more
semanticscholar   +1 more source

AAV-mediated factor IX gene transfer to skeletal muscle in patients with severe hemophilia B.

Blood, 2003
Hemophilia B is an X-linked coagulopathy caused by absence of functional coagulation factor IX (F.IX). Previously, we established an experimental basis for gene transfer as a method of treating the disease in mice and hemophilic dogs through ...
C. Manno, A. Chew, S. Hutchison, P. Larson, R. Herzog, V. Arruda, S. J. Tai, M. Ragni, A. Thompson, Margareth C. Ozelo, L. Couto, D. Leonard, F. Johnson, A. Mcclelland, C. Scallan, E. Skarsgard, A. Flake, M. Kay, K. High, B. Glader   +19 more
semanticscholar   +1 more source

Enhanced pharmacokinetic properties of a glycoPEGylated recombinant factor IX: a first human dose trial in patients with hemophilia B.

Blood, 2011
Replacement therapy with factor IX (FIX) concentrates is the recommended treatment for patients with hemophilia B, an X-linked bleeding disorder occurring in 1:25,000 male births.
C. Négrier, K. Knobe, A. Tiede, P. Giangrande, J. Møss   +4 more
semanticscholar   +1 more source

Scanning by DOVAM-S Detects All Unique Sequence Changes in Blinded Analyses: Evidence that the Scanning Conditions Are Generic

BioTechniques, 2000
The [detection of virtually all mutations]-SSCP (DOVAM-S) is a highly sensitive variant of single strand conformation polymorphism (SSCP). Mutations in the factor IX gene were used to find a set of five SSCP conditions that detects virtually all ...
C.H. Buzin, C.Y. Wen, V.Q. Nguyen, G. Nozari, A. Mengos, X. Li, J.S. Chen, Q. Liu, R.A. Gatti, F.K. Fujimura, S.S. Sommer   +10 more
doaj   +1 more source

Case Report: Severe hemophilia B patient with inhibitor and anaphylaxis reaction to FIX, successfully managed with concizumab prophylaxis therapy

Frontiers in Pediatrics
BackgroundHemophilia B is a rare X-linked disorder characterized by factor IX (FIX) deficiency, leading to spontaneous bleeding episodes predominantly affecting joints and muscles.
Elisa Bonetti, Maria Pia Esposto, Ada Zaccaron, Chiara Guardo, Giulia Caddeo, Matteo Chinello, Rita Balter, Vincenza Pezzella, Virginia Vitale, Simone Cesaro   +9 more
doaj   +1 more source

Prolonged activity of factor IX as a monomeric Fc fusion protein.

Blood, 2010
Treatment of hemophilia B requires frequent infusions of factor IX (FIX) to prophylax against bleeding episodes. Hemophilia B management would benefit from a FIX protein with an extended half-life.
R. Peters, S. Low, George D. Kamphaus, J. Dumont, J. Amari, Qi Lu, Gregory Zarbis-Papastoitsis, Tom J Reidy, E. Merricks, T. Nichols, A. Bitonti   +10 more
semanticscholar   +1 more source

THE USE OF EMICIZUMAB IN A CHILD WITH HEMOPHILIA A INHIBITORY FOREMA

Мать и дитя в Кузбассе, 2023
Hemophilia is a disease caused by hereditary deficiency of plasma coagulation factors VIII (FVIII) (hemophilia A) or IX (FIX) (hemophilia B) and characterized by hematomic bleeding.
Юлия Олеговна Рождественская, Анастасия Владимировна Миронова, Ирина Владимировна Болгова, Яна Вячеславовна Юнкина, Светлана Ивановна Елгина, Вадим Гельевич Мозес, Елена Владимировна Рудаева, Наталья Степановна Черных, Кира Борисовна Мозес, Яэль Центер   +9 more
doaj  

Factor IX administration in the skin primes inhibitor formation and sensitizes hemophilia B mice to systemic factor IX administration. [PDF]

Res Pract Thromb Haemost, 2023
Sherman A, Bertolini TB, Arisa S, Herzog RW, Kaczmarek R.   +4 more
europepmc   +1 more source

Biodistribution of recombinant factor IX, extended half-life recombinant factor IX Fc fusion protein, and glycoPEGylated recombinant factor IX in hemophilia B mice. [PDF]

Blood Coagul Fibrinolysis, 2023
van der Flier A, Hong V, Liu Z, Piepenhagen P, Ulinski G, Dumont JA, Orcutt KD, Goel A, Peters R, Salas J.   +9 more
europepmc   +1 more source

Hemofilia II. Aspectos moleculares y de genética poblacional Hemophilia II. Molecular and population genetics

Revista Cubana de Hematología, Inmunología y Hemoterapia, 2012
La prevalencia de la hemofilia esporádica fue estimada hace más de 40 años y se demostró que aproximadamente un tercio de los casos son de novo. La mayoría de las mutaciones que ocurren en la hemofilia se producen durante la espermatogénesis masculina ...
Dunia Castillo-González
doaj