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Von Willebrand factor processing

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Von Willebrand factor processing. / Brehm, Maria A.

In: HAMOSTASEOLOGIE, Vol. 37, No. 1, 31.01.2017, p. 59-72.

Research output: SCORING: Contribution to journal › SCORING: Review article › Research

Harvard

Brehm, MA 2017, 'Von Willebrand factor processing', HAMOSTASEOLOGIE, vol. 37, no. 1, pp. 59-72. https://doi.org/10.5482/HAMO-16-06-0018

APA

Brehm, M. A. (2017). Von Willebrand factor processing. HAMOSTASEOLOGIE, 37(1), 59-72. https://doi.org/10.5482/HAMO-16-06-0018

Vancouver

Brehm MA. Von Willebrand factor processing. HAMOSTASEOLOGIE. 2017 Jan 31;37(1):59-72. https://doi.org/10.5482/HAMO-16-06-0018

Bibtex

@article{395fbeafa6c84e3ca9e3911a08d50809,
title = "Von Willebrand factor processing",
abstract = "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.",
keywords = "Animals, Blood Platelets, Blood Vessels, Factor VIII, Humans, Immunity, Innate, Models, Cardiovascular, Models, Immunological, Platelet Activation, von Willebrand Factor, Journal Article, Review",
author = "Brehm, {Maria A}",
year = "2017",
month = jan,
day = "31",
doi = "10.5482/HAMO-16-06-0018",
language = "English",
volume = "37",
pages = "59--72",
journal = "HAMOSTASEOLOGIE",
issn = "0720-9355",
publisher = "Schattauer",
number = "1",

}

RIS

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 -