Weibel-Palade body
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In [[physiology]], '''Weibel-Palade bodies''' are [[organelle]]s in the [[endothelial cell]]s, the cells which make up the [[endothelium]], the exceedingly thin cell sheet which lines all [[blood vessel]]s, and the heart. They are [[eponym|named after]] the two [[scientist]]s who first described them in [[1964]]. They play a dual role in blood coagulation [[haemostasis]] and [[inflammation]].
==Constituents==
There are two major constituents of Weibel-Palade bodies. One is [[von Willebrand factor]] (vWF), a multimeric [[protein]] involved in [[blood]] [[coagulation]]<ref>{{cite journal |author=Wagner DD, Olmsted JB, Marder VJ |title=Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells |journal=J. Cell Biol. |volume=95 |issue=1 |pages=355–60 |year=1982 |month=October |pmid=6754744 |pmc=2112360 |url=http://www.jcb.org/cgi/reprint/95/1/355 | format=PDF |doi=10.1083/jcb.95.1.355}}</ref>. The second is [[P-selectin]]<ref>{{cite journal |author=Bonfanti R, Furie BC, Furie B, Wagner DD |title=PADGEM (GMP140) is a component of Weibel-Palade bodies of human endothelial cells |journal=Blood |volume=73 |issue=5 |pages=1109–12 |year=1989 |month=April |pmid=2467701 |url=http://bloodjournal.hematologylibrary.org/cgi/reprint/73/5/1109 | format=PDF}}</ref><ref>{{cite journal |author=McEver RP, Beckstead JH, Moore KL, Marshall-Carlson L, Bainton DF |title=GMP-140, a platelet alpha-granule membrane protein, is also synthesized by vascular endothelial cells and is localized in Weibel-Palade bodies |journal=J. Clin. Invest. |volume=84 |issue=1 |pages=92–9 |year=1989 |month=July |pmid=2472431 |pmc=303957 |doi=10.1172/JCI114175}}</ref>, which binds to passing immune cells ([[leukocytes]]). This allows the fast moving leukocytes in the blood to bind to the cells lining the blood vessels and slow down in a series of steps called the [[leukocyte adhesion cascade]]. Subsequently, leukocytes transmigrate across the [[endothelium]] and enter the surrounding tissue where they can migrate to the site of [[infection]].
Additional Weibel-Palade body components are the chemokines [[Interleukin-8]] and [[eotaxin-3]], [[endothelin-1]], [[angiopoietin-2]], [[osteoprotegerin]], the [[tetraspanin]] CD63/lamp3 and α-1,3-fucosyltransferase VI.
==Clinical significance==
The importance of Weibel-Palade bodies are highlighted by some human disease mutations. Mutations within vWF are the usual cause of the most common inherited bleeding disorder, [[von Willebrand disease]]. VWD has an estimated prevalence in some human populations of up to 1%, and is most often characterised by prolonged and variable [[mucocutaneous]] bleeding. Type III von Willebrand Disease is a severe bleeding disorder, not unlike severe [[haemophilia]] type A or B. VWF acts in primary [[haemostasis]] to recruit [[platelets]] at a site of injury, and is also important in secondary [[hemostasis]], acting as a chaperone for [[coagulation]] factor VIII (FVIII).
==Production==
Multimeric vWF is assembled in the [[Golgi apparatus]] from vWF dimers. The Golgi then buds off vesicles, covered in a [[lipid bilayer]], which consist almost exclusively of vWF. The only parallel organelle in physiology is the ''alpha'' granule of [[platelet]]s, which also contains vWF. Weibel-Palade bodies are the main source of vWF, though, and α-granules probably play a minor role.
==History==
Weibel-Palade bodies were initially described by the [[Switzerland|Swiss]] [[anatomy|anatomist]] [[Ewald R. Weibel]] and the [[Romanians|Romanian]] [[physiology|physiologist]] [[George Emil Palade]] in [[1964]].<ref>{{cite journal |author=Weibel ER, Palade GE |title=New cytoplasmic components in arterial endothelia |journal=J. Cell Biol. |volume=23 |issue= |pages=101–12 |year=1964 |month=October |pmid=14228505 |pmc=2106503 |doi=10.1083/jcb.23.1.101}}</ref> Prof. Palade was to win the [[Nobel Prize in Physiology or Medicine]] in [[1974]] for his work on the function of organelles in [[cell (biology)|cells]].
==References==
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==See also==
* [[Endothelium]]
* [[Von Willebrand factor]]
* [[Platelet]]
==External links==
* {{MeshNumber|A11.284.195.190.875.190.190.950|Weibel-Palade+Bodies}}
[[Category:Organelles]]
[[de:Weibel-Palade-Körperchen]]