3.Laboratory assessment of von Willebrand factor for classification of von Willebrand disease.
Young Woo SON ; Kyung Chae KYE ; Hyun Chun SHIN ; Hong Bock LEE ; Do Yeun OH ; Seon Yang PARK ; Byeong Kook KIM ; Noe Kyeong KIM
Korean Journal of Hematology 1993;28(2):345-350
No abstract available.
Classification*
;
von Willebrand Diseases*
;
von Willebrand Factor*
4.Type II variant von Willebrand disease in a family.
Chang Hoon LEE ; Kyung Soon SONG ; Jong Hwa LEE ; Won Bae KIM ; Duk Yong KANG ; Chan Sub SIM
Korean Journal of Clinical Pathology 1992;12(3):325-330
No abstract available.
Humans
;
von Willebrand Diseases*
5.Type II variant von Willebrand disease in a family.
Chang Hoon LEE ; Kyung Soon SONG ; Jong Hwa LEE ; Won Bae KIM ; Duk Yong KANG ; Chan Sub SIM
Korean Journal of Clinical Pathology 1992;12(3):325-330
No abstract available.
Humans
;
von Willebrand Diseases*
6.von Willebrand Disease.
Korean Journal of Hematology 1999;34(1):1-7
No abstract available.
von Willebrand Diseases*
7.Three cases of type I von Willebrand disease in a family.
Jie Sun YOON ; Hey Sun LEE ; Young Min AHN ; Myoung Sook KOO ; Sun Yang PARK
Journal of the Korean Pediatric Society 1991;34(3):419-425
No abstract available.
Humans
;
von Willebrand Disease, Type 1*
;
von Willebrand Diseases
8.Three cases of type I von Willebrand disease in a family.
Jie Sun YOON ; Hey Sun LEE ; Young Min AHN ; Myoung Sook KOO ; Sun Yang PARK
Journal of the Korean Pediatric Society 1991;34(3):419-425
No abstract available.
Humans
;
von Willebrand Disease, Type 1*
;
von Willebrand Diseases
9.Research advance in von Willebrand factor.
Xue-Mei LI ; Miao JIANG ; Yi-Ming ZHAO
Journal of Experimental Hematology 2013;21(3):801-805
von Willebrand factor (vWF) is a multimeric glycoprotein exclusively synthesized in endothelial cells and megakaryocytes. It plays important roles in the primary and secondary haemostasis. Deficiency or dysfunction of vWF may cause von Willebrand disease (vWD), and overexpression of vWF may cause thrombosis. Making an intensive study on vWF will help us to understand the pathophysiology, diagnosis and treatment of vWF-related diseases, such as vWD, TTP, venous thrombosis, stroke, and so on. In this article, the regulation of vWF activity and its relation with diseases mentioned above are reviewed.
Humans
;
von Willebrand Diseases
;
blood
;
pathology
;
von Willebrand Factor
10.The Effect of VWF Propeptide on VWF Mutant in D1 Domain.
Xiu-Qun YU ; Zhen-Ni MA ; Jing LING ; Yun-Xiao ZHAO ; Jie YIN ; Zi-Qiang YU ; Chang-Geng RUAN
Journal of Experimental Hematology 2022;30(5):1541-1548
OBJECTIVE:
To investigate whether co-transfection of wild-type VWFpp with VWF mutant in D1 region is able to correct VWF defects in biosynthesis and secretion.
METHODS:
Four VWF mutant plasmids were single transfected into HEK 293 cells, or co-transfected into HEK 293 cells with the wild type VWFpp plasmids. The VWF in supernatant and lysate of transfected cells were analyzed by ELISA, vertical VWF multimer electrophoresis. The retention of VWF in endoplasmic reticulum of transfected cells were detected by immunofluorescence confocal microscope.
RESULTS:
In the vertical VWF multimer analysis, with co-expressing VWF mutant and VWFpp, the VWF multimer bands disappeared, and the VWF antigen in both supernatant and lysate of cells decreased, compared with the single expression of VWF mutant. Although the intracellular levels of VWF antigens decreased after co-expression, the retention rate of VWF mutant decreased in endoplasmic reticulum.
CONCLUSION
VWFpp can reduce the retention of VWF in endoplasmic reticulum, assists the transport of VWF between subcellular organelles. However, VWFpp inhibits the biosynthesis and secretion of VWF about the mutant in D1 domain.
HEK293 Cells
;
Humans
;
von Willebrand Diseases
;
von Willebrand Factor/metabolism*
Result Analysis
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