Hemophilia A and B induce recurrent bleeding episodes, mainly in skeletal muscles and joints that are in intermittent motion. We have previously demonstrated that intermittent motion contributes to increased degradation of factors VIII and IX. Given that calcium ions are known to enhance factor VIII-von Willebrand factor (vWF) interaction, the present study has investigated the role of these ions on factors VIII and IX in the condition of motion. The effects of calcium ions were assessed using purified proteins via Western blot, factor VIII activity, immunocytochemistry, and in Institute of Cancer Research (ICR) mice with no specific genetic background. Calcium was found to prevent degradation of plasma-derived factor VIII but not that of factor IX, during intermittent motion. Calcium levels in the microcirculation of mouse striated muscles were elevated following movement, enabling prevention of factor VIII degradation in normal physiology. Calcium supplementation in drinking water increased factor VIII levels in blood and striated muscles of ICR mice during movement. calcium ions decrease factor VIII degradation in the condition of motion. Further research on the impact of calcium salt oral supplementation on hemophilia patients is warranted.
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http://dx.doi.org/10.3390/biology12111388 | DOI Listing |
Cureus
December 2024
Ophthalmology, Palmetto Retina Center, Columbia, USA.
The purpose of this manuscript is to report a rare case of pediatric central retinal artery occlusion (CRAO) in the setting of atypical hypercoagulable tests. An 11-year-old female presented to the emergency department with painless, visual changes in the left eye. Ophthalmological examination was remarkable for a central area of retinal ischemia and edema with sparing along the distribution of the cilioretinal artery along with a cherry red spot, all of which were consistent with a CRAO.
View Article and Find Full Text PDFJ Phys Chem B
January 2025
Chemistry Department, Western Washington University, Bellingham, Washington 98225-9038, United States.
During the blood coagulation cascade, coagulation factor VIII (FVIII) is activated by thrombin to form activated factor VIII (FVIIIa). FVIIIa associates with platelet surfaces at the site of vascular damage to form an intrinsic tenase complex with activated factor IX. A working model for FVIII membrane binding involves the association of positively charged FVIII residues with negatively charged lipid headgroups and the burial of hydrophobic residues into the membrane interior.
View Article and Find Full Text PDFJ Toxicol Pathol
January 2025
The Institute of Environmental Toxicology, 4321 Uchimoriya-machi, Joso-shi, Ibaraki 303-0043, Japan.
Cystic degeneration (CD) in the liver is a cyst-like lesion composed of one or more pseudocysts lacking lining cells, occurring spontaneously in rats older than 12 months, with a male predilection. In this study, 32 CDs were identified in 23 out of 104 non-treated, control male Sprague-Dawley rats from two combined chronic toxicity and carcinogenicity studies with agrochemicals. They were examined histologically, histochemically, and immunohistochemically to assess the pathogenesis and pathological significance of CD, focusing on pseudocapillarization in aged rat liver.
View Article and Find Full Text PDFThromb Haemost
January 2025
Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.
Background: We previously identified a factor (F)VIII molecular defect associated with an R2159C mutation in the C1 domain (named "FVIII-Ise") together with undetectable FVIII antigen (FVIII:Ag) levels measured by two-site sandwich ELISA using an anti-C2 domain alloantibody (alloAb). The patient had clinically mild hemophilia A, and his reduced FVIII:C correlated with FVIII:Ag measured by ELISA using monoclonal antibodies (mAbs) with A2 and A2/B domain epitopes, suggesting that the R2159C mutation modified C2 domain antigenicity.
Aim: To investigate functional and structural characteristics of the FVIII-R2159C mutant.
Hematol Oncol Stem Cell Ther
January 2025
Department of Hematology, Ankara University School of Medicine, Ankara, Turkey.
Despite numerous reports on the procoagulant activities of G-CSF, the effect of plerixafor on the hemostatic system is not clearly understood. This study aims to evaluate the effects of plerixafor on the hemostatic system when used for autologous stem cell mobilization (ASCM) for poor mobilizers (PM) with lymphoma and multiple myeloma. Patients who were performed ASCM with plerixafor in combination with GCSF were prospectively enrolled.
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