A novel inducible von Willebrand Factor Cre recombinase mouse strain to study microvascular endothelial cell-specific biological processes in vivo
Vascul Pharmacol. 2024 Mar 28:107369. doi: 10.1016/j.vph.2024.107369. Online ahead of print.ABSTRACTMouse models are invaluable to understanding fundamental mechanisms in vascular biology during development, in health and different disease states. Several constitutive or inducible models that selectively knockout or knock in genes in vascular endothelial cells exist; however, functional and phenotypic differences exist between microvascular and macrovascular endothelial cells in different organs. In order to study microvascular endothelial cell-specific biological processes, we developed a Tamoxifen-inducible von Willebran...
Source: Vascular Pharmacology - March 30, 2024 Category: Drugs & Pharmacology Authors: Dinesh Yadav Jeremy A Conner Yimin Wang Thomas L Saunders Eroboghene E Ubogu Source Type: research

A novel inducible von Willebrand Factor Cre recombinase mouse strain to study microvascular endothelial cell-specific biological processes in vivo
Vascul Pharmacol. 2024 Mar 28:107369. doi: 10.1016/j.vph.2024.107369. Online ahead of print.ABSTRACTMouse models are invaluable to understanding fundamental mechanisms in vascular biology during development, in health and different disease states. Several constitutive or inducible models that selectively knockout or knock in genes in vascular endothelial cells exist; however, functional and phenotypic differences exist between microvascular and macrovascular endothelial cells in different organs. In order to study microvascular endothelial cell-specific biological processes, we developed a Tamoxifen-inducible von Willebran...
Source: Vascular Pharmacology - March 30, 2024 Category: Drugs & Pharmacology Authors: Dinesh Yadav Jeremy A Conner Yimin Wang Thomas L Saunders Eroboghene E Ubogu Source Type: research

A novel inducible von Willebrand Factor Cre recombinase mouse strain to study microvascular endothelial cell-specific biological processes in vivo
Vascul Pharmacol. 2024 Mar 28:107369. doi: 10.1016/j.vph.2024.107369. Online ahead of print.ABSTRACTMouse models are invaluable to understanding fundamental mechanisms in vascular biology during development, in health and different disease states. Several constitutive or inducible models that selectively knockout or knock in genes in vascular endothelial cells exist; however, functional and phenotypic differences exist between microvascular and macrovascular endothelial cells in different organs. In order to study microvascular endothelial cell-specific biological processes, we developed a Tamoxifen-inducible von Willebran...
Source: Vascular Pharmacology - March 30, 2024 Category: Drugs & Pharmacology Authors: Dinesh Yadav Jeremy A Conner Yimin Wang Thomas L Saunders Eroboghene E Ubogu Source Type: research

A novel inducible von Willebrand Factor Cre recombinase mouse strain to study microvascular endothelial cell-specific biological processes in vivo
Vascul Pharmacol. 2024 Mar 29;155:107369. doi: 10.1016/j.vph.2024.107369. Online ahead of print.ABSTRACTMouse models are invaluable to understanding fundamental mechanisms in vascular biology during development, in health and different disease states. Several constitutive or inducible models that selectively knockout or knock in genes in vascular endothelial cells exist; however, functional and phenotypic differences exist between microvascular and macrovascular endothelial cells in different organs. In order to study microvascular endothelial cell-specific biological processes, we developed a Tamoxifen-inducible von Wille...
Source: Vascular Pharmacology - March 30, 2024 Category: Drugs & Pharmacology Authors: Dinesh Yadav Jeremy A Conner Yimin Wang Thomas L Saunders Eroboghene E Ubogu Source Type: research

A novel inducible von Willebrand Factor Cre recombinase mouse strain to study microvascular endothelial cell-specific biological processes in vivo
Vascul Pharmacol. 2024 Mar 29;155:107369. doi: 10.1016/j.vph.2024.107369. Online ahead of print.ABSTRACTMouse models are invaluable to understanding fundamental mechanisms in vascular biology during development, in health and different disease states. Several constitutive or inducible models that selectively knockout or knock in genes in vascular endothelial cells exist; however, functional and phenotypic differences exist between microvascular and macrovascular endothelial cells in different organs. In order to study microvascular endothelial cell-specific biological processes, we developed a Tamoxifen-inducible von Wille...
Source: Vascular Pharmacology - March 30, 2024 Category: Drugs & Pharmacology Authors: Dinesh Yadav Jeremy A Conner Yimin Wang Thomas L Saunders Eroboghene E Ubogu Source Type: research

Hereditary TTP/Upshaw-Schulman syndrome: the ductus arteriosus controls newborn survival
Int J Hematol. 2024 Mar 27. doi: 10.1007/s12185-024-03731-1. Online ahead of print.ABSTRACTHereditary TTP (hTTP), termed Upshaw-Schulman syndrome, is an ultra-rare disorder caused by a severe deficiency of plasma ADAMTS13 activity that allows circulation of ultra-large von Willebrand factor (UL-VWF) multimers. The greatest risk for hTTP is in their first days after birth, when 35-50% of patients will have severe hemolysis, jaundice, and thrombocytopenia. It is often fatal without effective treatment. In utero, fetal blood flowing from the pulmonary artery through the ductus arteriosus (DA) to the aorta is under low-shear-f...
Source: International Journal of Hematology - March 27, 2024 Category: Hematology Authors: Yoshihiro Fujimura Source Type: research

Hereditary TTP/Upshaw-Schulman syndrome: the ductus arteriosus controls newborn survival
Int J Hematol. 2024 Mar 27. doi: 10.1007/s12185-024-03731-1. Online ahead of print.ABSTRACTHereditary TTP (hTTP), termed Upshaw-Schulman syndrome, is an ultra-rare disorder caused by a severe deficiency of plasma ADAMTS13 activity that allows circulation of ultra-large von Willebrand factor (UL-VWF) multimers. The greatest risk for hTTP is in their first days after birth, when 35-50% of patients will have severe hemolysis, jaundice, and thrombocytopenia. It is often fatal without effective treatment. In utero, fetal blood flowing from the pulmonary artery through the ductus arteriosus (DA) to the aorta is under low-shear-f...
Source: International Journal of Hematology - March 27, 2024 Category: Hematology Authors: Yoshihiro Fujimura Source Type: research

A Rare Case of Klippel Trenaunay Syndrome with Von Willebrand Factor Deficiency and Multiple Accessory Spleens: A Case Report and Brief Literature Review
We present a 13-year-old son with a typical clinical manifestation of KTS, including "port-wine stains" as capillary malformation, venous malformation, and hypertrophy of the left lower extremity, who also suffers from von Willebrand Disease type 3. He has been suffering from these two rare conditions since birth. The occurrence of KTS with von Willebrand Factor deficiency in a patient has so far not been reported, which may propose a mutation in the putative common regulatory gene that caused this uncommon phenotype.PMID:38525403 | PMC:PMC10958732 | DOI:10.4103/abr.abr_232_23 (Source: Biomed Res)
Source: Biomed Res - March 25, 2024 Category: Research Authors: Vahid Falahati Mahsa Fallahi Mona Shahriarpour Ali Ghasemi Kazem Ghaffari Source Type: research

A Rare Case of Klippel Trenaunay Syndrome with Von Willebrand Factor Deficiency and Multiple Accessory Spleens: A Case Report and Brief Literature Review
We present a 13-year-old son with a typical clinical manifestation of KTS, including "port-wine stains" as capillary malformation, venous malformation, and hypertrophy of the left lower extremity, who also suffers from von Willebrand Disease type 3. He has been suffering from these two rare conditions since birth. The occurrence of KTS with von Willebrand Factor deficiency in a patient has so far not been reported, which may propose a mutation in the putative common regulatory gene that caused this uncommon phenotype.PMID:38525403 | PMC:PMC10958732 | DOI:10.4103/abr.abr_232_23 (Source: Biomed Res)
Source: Biomed Res - March 25, 2024 Category: Research Authors: Vahid Falahati Mahsa Fallahi Mona Shahriarpour Ali Ghasemi Kazem Ghaffari Source Type: research

Inhibition of platelet adhesion to exposed subendothelial collagen by steric hindrance with blocking peptide nanoparticles
This study aimed to engineer peptide-based nanoparticles that prevent platelet binding to subendothelial collagen by engaging with collagen with high affinity. We examined the interactions between integrin α2/ glycoprotein VI/ von Willebrand factor A3 domain and collagen, as well as between the synthesized peptide nanoparticles and collagen, utilizing molecular dynamics simulations and empirical assays. Our findings indicated that the bond between von Willebrand factor and collagen was more robust. Specifically, the sequences SITTIDV, VDVMQRE, and YLTSEMH in von Willebrand factor were identified as essential for its attac...
Source: Colloids and Surfaces - March 23, 2024 Category: Biotechnology Authors: Anqi Wang Kai Yue Xiaotong Yan Weishen Zhong Genpei Zhang Lei Wang Hua Zhang Xinxin Zhang Source Type: research

Inhibition of platelet adhesion to exposed subendothelial collagen by steric hindrance with blocking peptide nanoparticles
This study aimed to engineer peptide-based nanoparticles that prevent platelet binding to subendothelial collagen by engaging with collagen with high affinity. We examined the interactions between integrin α2/ glycoprotein VI/ von Willebrand factor A3 domain and collagen, as well as between the synthesized peptide nanoparticles and collagen, utilizing molecular dynamics simulations and empirical assays. Our findings indicated that the bond between von Willebrand factor and collagen was more robust. Specifically, the sequences SITTIDV, VDVMQRE, and YLTSEMH in von Willebrand factor were identified as essential for its attac...
Source: Atherosclerosis - March 23, 2024 Category: Cardiology Authors: Anqi Wang Kai Yue Xiaotong Yan Weishen Zhong Genpei Zhang Lei Wang Hua Zhang Xinxin Zhang Source Type: research

Inhibition of platelet adhesion to exposed subendothelial collagen by steric hindrance with blocking peptide nanoparticles
This study aimed to engineer peptide-based nanoparticles that prevent platelet binding to subendothelial collagen by engaging with collagen with high affinity. We examined the interactions between integrin α2/ glycoprotein VI/ von Willebrand factor A3 domain and collagen, as well as between the synthesized peptide nanoparticles and collagen, utilizing molecular dynamics simulations and empirical assays. Our findings indicated that the bond between von Willebrand factor and collagen was more robust. Specifically, the sequences SITTIDV, VDVMQRE, and YLTSEMH in von Willebrand factor were identified as essential for its attac...
Source: Colloids and Surfaces - March 23, 2024 Category: Biotechnology Authors: Anqi Wang Kai Yue Xiaotong Yan Weishen Zhong Genpei Zhang Lei Wang Hua Zhang Xinxin Zhang Source Type: research

O blood type is not associated with worse coagulopathy or outcome in exsanguinating trauma
Despite improving understanding of trauma-induced coagulopathy (TIC), mortality and morbidity due to exsanguinating trauma remain high. Increased complications due to hemorrhage have been reported in blood group O, possibly due to reduced levels of von Willebrand factor (vWF). (Source: American Journal of Surgery)
Source: American Journal of Surgery - March 23, 2024 Category: Surgery Authors: J. Cole Gwin, Niyati Rangnekar, Glenn P. Murray, Saskya Bylerly, Andrew M. Fleming, Thomas S. Easterday, Andrew J. Kerwin, Isaac W. Howley Tags: Original Research Article Source Type: research

Use of Vonicog Alpha and Acquired von Willebrand Syndrome, a New Approach: A Case Report
Hamostaseologie. 2024 Mar 22. doi: 10.1055/a-2266-7984. Online ahead of print.ABSTRACTTherapeutic management of acquired von Willebrand syndrome (AVWS) can be challenging, particularly in cases of AVWS associated with monoclonal IgM such as Waldenström macroglobulinemia (WM) where several therapeutic options may be ineffective. Here, we describe the case of an 88-year-old patient who developed AVWS during follow-up for WM. The presence of a severe bleeding symptomatology not controlled by several therapies (plasma-derived von Willebrand factor, plasmapheresis) led us to introduce a supplementation with recombinant von Wil...
Source: Hamostaseologie - March 22, 2024 Category: Hematology Authors: Adeline Blandini ères Sophie Combe No émie Chanson Olivier Lambotte C écile Lavenu-Bombled Source Type: research