A new method for detection of inhibitors against factor XIII is described. Agarose gel electrophoresis with normal platelet poor plasma as a factor XIII source included in the gel was combined with reversed activity staining. The procedure was applied to a 10-year-old girl with factor XIII deficiency where substitution therapy had failed. Plasma samples from the patient inhibited the factor XIII catalysed incorporation of the fluorescent monodansylthiacadaverine into casein at a position corresponding to the migration of gamma-globulins. Treatment with Sepharose-bound protein A effectively adsorbed the factor XIII antibody in the patient's plasma.
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http://dx.doi.org/10.1111/j.1365-2141.1992.tb06438.x | DOI Listing |
J Thromb Haemost
January 2025
Department of Medicine, McMaster University; Department of Biochemistry and Biomedical Sciences, McMaster University; Thrombosis and Atherosclerosis Research Institute, McMaster University and Hamilton Health Sciences.
Thrombin is the central mediator of hemostasis, where it converts fibrinogen to fibrin, activates upstream factors to promote coagulation, activates factor XIII and thrombin-activatable fibrinolysis inhibitor to stabilize fibrin, mediates anticoagulation, and modulates cellular activity via cell surface receptors. Thus, regulation of thrombin activity is essential to the hemostatic balance. Thrombin is regulated by positively charged surface domains that surround the active site.
View Article and Find Full Text PDFHematol Oncol Stem Cell Ther
January 2025
Adult Hematology, Stem Cell Transplant and Cellular Therapy Section, Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Acquired factor XIII (FXIII) deficiency is a rare disorder that could be associated with autoimmune and malignant disorders with a high risk of bleeding. In acute leukemias, acquired FXIII deficiency has been reported and replacement of FXIII helped to control significant bleeding. Here, we report four cases of myeloid neoplasms to have acquired FXIII deficiency with interesting concomitant RUNX1 mutation in the molecular background of two patients.
View Article and Find Full Text PDFPoult Sci
January 2025
College of Animal Science and Technology, Henan Agricultural University, Zhengzhou 450046, China; International Joint Research Laboratory for Poultry Breeding of Henan, Zhengzhou, 450046, China. Electronic address:
The transglutaminase (TGMs) family plays a crucial role in regulating mammalian reproduction, yet its impact on poultry reproductive traits has not been extensively studied. In this study, we identified eight members of the TGMs family in chickens and examined the contributions of genetic variations of coagulation factor XIII A chain (F13A1), transglutaminase 4 (TGM4), and LOC101749664 to selective breeding in commercial layers through genetic variation response pattern analysis. Transcriptome data from various tissues of high- and low-egg-yielding Gushi chickens revealed significant positive correlations between the mRNA expression levels of TGM4 and F13A1 genes and egg production (P < 0.
View Article and Find Full Text PDFBlood Transfus
December 2024
Department of Anesthesiology and Intensive Care Medicine, University Hospital Center Rijeka, Rijeka, Croatia.
CEN Case Rep
January 2025
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
A 69-year-old Japanese man developed abdominal pain, purpura, proteinuria, and hematuria while receiving treatment for pulmonary tuberculosis. A skin biopsy revealed IgA-positive leukocytoclastic vasculitis, and a renal biopsy showed IgA-positive mesangial proliferative glomerulonephritis with crescent formation. Based on these findings, we diagnosed IgA vasculitis with nephritis (IgAVN) and initiated treatment.
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