Von Willebrand factor processing
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Von Willebrand factor processing. / Brehm, Maria A.
in: HAMOSTASEOLOGIE, Jahrgang 37, Nr. 1, 31.01.2017, S. 59-72.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung
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TY - JOUR
T1 - Von Willebrand factor processing
AU - Brehm, Maria A
PY - 2017/1/31
Y1 - 2017/1/31
N2 - Von Willebrand factor (VWF) is a multimeric glycoprotein essential for primary haemostasis that is produced only in endothelial cells and megakaryocytes. Key to VWF's function in recruitment of platelets to the site of vascular injury is its multimeric structure. The individual steps of VWF multimer biosynthesis rely on distinct posttranslational modifications at specific pH conditions, which are realized by spatial separation of the involved processes to different cell organelles. Production of multimers starts with translocation and modification of the VWF prepropolypeptide in the endoplasmic reticulum to produce dimers primed for glycosylation. In the Golgi apparatus they are further processed to multimers that carry more than 300 complex glycan structures functionalized by sialylation, sulfation and blood group determinants. Of special importance is the sequential formation of disulfide bonds with different functions in structural support of VWF multimers, which are packaged, stored and further processed after secretion. Here, all these processes are being reviewed in detail including background information on the occurring biochemical reactions.
AB - Von Willebrand factor (VWF) is a multimeric glycoprotein essential for primary haemostasis that is produced only in endothelial cells and megakaryocytes. Key to VWF's function in recruitment of platelets to the site of vascular injury is its multimeric structure. The individual steps of VWF multimer biosynthesis rely on distinct posttranslational modifications at specific pH conditions, which are realized by spatial separation of the involved processes to different cell organelles. Production of multimers starts with translocation and modification of the VWF prepropolypeptide in the endoplasmic reticulum to produce dimers primed for glycosylation. In the Golgi apparatus they are further processed to multimers that carry more than 300 complex glycan structures functionalized by sialylation, sulfation and blood group determinants. Of special importance is the sequential formation of disulfide bonds with different functions in structural support of VWF multimers, which are packaged, stored and further processed after secretion. Here, all these processes are being reviewed in detail including background information on the occurring biochemical reactions.
KW - Animals
KW - Blood Platelets
KW - Blood Vessels
KW - Factor VIII
KW - Humans
KW - Immunity, Innate
KW - Models, Cardiovascular
KW - Models, Immunological
KW - Platelet Activation
KW - von Willebrand Factor
KW - Journal Article
KW - Review
U2 - 10.5482/HAMO-16-06-0018
DO - 10.5482/HAMO-16-06-0018
M3 - SCORING: Review article
C2 - 28139814
VL - 37
SP - 59
EP - 72
JO - HAMOSTASEOLOGIE
JF - HAMOSTASEOLOGIE
SN - 0720-9355
IS - 1
ER -