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invest with or as with a body; give body to (同)personify
the external structure of a vehicle; "the body of the car was badly rusted"
the main mass of a thing
a natural object consisting of a dead animal or person; "they found the body in the lake" (同)dead body
the entire structure of an organism (an animal, plant, or human being); "he felt as if his whole body were on fire" (同)organic structure, physical structure
a collection of particulars considered as a system; "a body of law"; "a body of doctrine"; "a body of precedents"
a group of persons associated by some common tie or occupation and regarded as an entity; "the whole body filed out of the auditorium"; "the student body"; "administrative body"
an individual 3-dimensional object that has mass and that is distinguishable from other objects; "heavenly body"
the central message of a communication; "the body of the message was short"

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〈C〉『身体』,肉体 / 〈C〉(人・動物の)『胴体』 / 〈C〉)物の)『主要部』,本体《+『of』+『名』》 / 〈C〉(…の)『団体』,群れ:(…のたくさんの)集まり《+『of』+『名』》 / 〈C〉物体,…体 / 〈U〉実質;(酒・味などの)こく / 〈C〉《話》人
《英話》人;(特に)男

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/09/19 08:33:55」(JST)

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Weibel–Palade bodies are the storage granules of endothelial cells, the cells that form the inner lining of the blood vessels and heart. They store and release two principal molecules, von Willebrand factor and P-selectin, and thus play a dual role in hemostasis and inflammation.

Etymology

Weibel–Palade bodies were initially described by the Swiss anatomist Ewald R. Weibel and the Romanian physiologist George Emil Palade in 1964.^[1] Palade won Nobel Prize in Physiology and Medicine in 1974 for his work on the function of organelles in cells.

Constituents

There are two major components stored within Weibel–Palade bodies. One is von Willebrand factor (vWF), a multimeric protein that plays a major role in blood coagulation.^[2] Storage of long polymers of vWF gives this specialized lysosomal structure an oblong shape and striated appearance on electron microscope.^[3] The other is P-selectin,^[4]^[5] which plays a central role in the ability of inflamed endothelial cells to recruit passing leukocytes (white blood cells), allowing them to exit the blood vessel (extravasate) and enter the surrounding tissue, where they can migrate to the site of infection or injury.

Additional Weibel–Palade body components are the chemokines Interleukin-8 and eotaxin-3, endothelin-1, angiopoietin-2, osteoprotegerin, the P-selectin cofactor CD63/lamp3,^[6] and α-1,3-fucosyltransferase VI.

Production

Multimeric vWF is assembled head-to-head in the Golgi apparatus from tail-to-tail vWF dimers. vWF multimers condense and twist into long, helical, mostly parallel tubules separated by a less dense matrix of protein domains protruding from the tubules.^[7] The Golgi then buds off clathrin-coated vesicles which consist almost exclusively of vWF.

Immature Weibel–Palade bodies remain near the nucleus, where they acquire more membrane proteins and then disperse throughout the cytoplasm, carried along microtubules by kinesins.^[6] Clathrin-coated vesicles bud from immature Weibel–Palade bodies, reducing their volumes, condensing their contents, and removing select membrane proteins. Maturing Weibel–Palade bodies may also fuse with each other.^[7]

The only parallel organelle in physiology is the alpha granule of platelets, which also contains vWF. Weibel–Palade bodies are the main source of vWF, while α-granules probably play a minor role.

Secretion

The small subset of Weibel–Palade bodies tethered at the cell periphery to the actin cortex serve as a readily releasable pool that's replenished by a larger pool of microtubule-associated bodies in the cell interior.^[6]

The contents of Weibel–Palade bodies are secreted by one of three mechanisms.^[7] Some undergo exocytosis individually, while others fuse transiently to the plasma membrane in a "lingering kiss" that opens a pore large enough for only their smaller cargo (e.g. IL-8, CD63) to diffuse out.^[7] Weibel–Palade bodies may also coalesce into larger vesicles called secretory pods for multigranular exocytosis.^[7] Secretory pod formation is mediated by interposition of tiny nanovesicles between bodies. As Weibel–Palade bodies fuse with secretory pods, their vWF cargo loses its tubular form for spaghetti-like strings that are then exocytosed through a fusion pore.^[7] Whether cargo besides vWF is exocytosed from secretory pods or selectively retained is uncertain. Different modes of cargo release from Weibel–Palade bodies may be a mechanism for differential release of subsets of molecules in different physiological conditions.^[7]

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 characterized by prolonged and variable mucocutaneous bleeding. Type III von Willebrand Disease is a severe bleeding disorder, not unlike severe hemophilia type A or B. VWF acts in primary hemostasis to recruit platelets at a site of injury, and is also important in secondary hemostasis, acting as a chaperone for coagulation factor VIII (FVIII).

References

^ Weibel ER, Palade GE; Palade (October 1964). "New cytoplasmic components in arterial endothelia". J. Cell Biol. 23 (1): 101–12. doi:10.1083/jcb.23.1.101. PMC 2106503. PMID 14228505.
^ Wagner, D. D.; Olmsted, J. B.; Marder, V. J. (October 1982). "Immunolocalization of von Willebrand protein in Weibel–Palade bodies of human endothelial cells" (PDF). J. Cell Biol. 95 (1): 355–60. doi:10.1083/jcb.95.1.355. PMC 2112360. PMID 6754744.
^ Tuma, Ronald F.; Durán, Walter N.; Ley, Klaus, eds. (2008). Microcirculation (2nd ed.). Amsterdam: Elsevier/Academic Press. p. 38. ISBN 978-0-12-374530-9.
^ Bonfanti, R.; Furie, B. C.; Furie, B.; Wagner, D. D. (1 April 1989). "PADGEM (GMP140) is a component of Weibel–Palade bodies of human endothelial cells" (PDF). Blood 73 (5): 1109–12. PMID 2467701.
^ McEver, R. P.; Beckstead, J. H.; Moore, K. L.; Marshall-Carlson, L.; Bainton, D. F. (July 1989). "GMP-140, a platelet alpha-granule membrane protein, is also synthesized by vascular endothelial cells and is localized in Weibel–Palade bodies". J. Clin. Invest. 84 (1): 92–9. doi:10.1172/JCI114175. PMC 303957. PMID 2472431.
^ ^a ^b ^c Doyle, E. L.; Ridger, V.; Ferraro, F.; Turmaine, M.; Saftig, P.; Cutler, D. F. (Oct 13, 2011). "CD63 is an essential cofactor to leukocyte recruitment by endothelial P-selectin". Blood 118 (15): 4265–73. doi:10.1182/blood-2010-11-321489. PMID 21803846.
^ ^a ^b ^c ^d ^e ^f ^g Valentijn KM, Sadler JE, Valentijn JA, Voorberg J, Eikenboom J (May 12, 2011). "Functional architecture of Weibel-Palade bodies". Blood 117 (19): 5033–5043. doi:10.1182/blood-2010-09-267492. PMID 21266719.

External links

MeSH A11.284.195.190.875.190.190.950

UpToDate Contents

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1. von Willebrand因子の生物学および正常機能 biology and normal function of von willebrand factor
2. 悪性腫瘍に関連した凝固亢進状態の病因 pathogenesis of the hypercoagulable state associated with malignancy

English Journal

The association of the Thr715Pro P-selectin genotype with levels of P-selectin in platelet concentrates.

Jilma-Stohlawetz P1, Mannhalter C, Kaider A, Waidacher T, Jilma B, Panzer S.
Vox sanguinis.Vox Sang.2014 Jun 30. doi: 10.1111/vox.12175. [Epub ahead of print]
BACKGROUND AND OBJECTIVES: P-selectin is stored in the alpha granules of platelets and in the Weibel Palade bodies of endothelial cells; upon activation, it is translocated to the cell surface and released into the plasma in soluble form. One variant of the P-selectin gene, the Thr715Pro polymorphis
PMID 24980694

STXBP1 promotes Weibel-Palade body exocytosis through its interaction with the Rab27A effector Slp4-a.

van Breevoort D1, Snijders AP2, Hellen N3, Weckhuysen S4, van Hooren KW1, Eikenboom J5, Valentijn K6, Fernandez-Borja M7, Ceulemans B8, De Jonghe P4, Voorberg J1, Hannah M9, Carter T10, Bierings R11.
Blood.Blood.2014 May 15;123(20):3185-94. doi: 10.1182/blood-2013-10-535831. Epub 2014 Apr 3.
Vascular endothelial cells contain unique rod-shaped secretory organelles, called Weibel-Palade bodies (WPBs), which contain the hemostatic protein von Willebrand factor (VWF) and a cocktail of angiogenic and inflammatory mediators. We have shown that the Rab27A effector synaptotagmin-like protein 4
PMID 24700782

A two-tier Golgi-based control of organelle size underpins the functional plasticity of endothelial cells.

Ferraro F1, Kriston-Vizi J2, Metcalf DJ3, Martin-Martin B1, Freeman J4, Burden JJ5, Westmoreland D1, Dyer CE1, Knight AE3, Ketteler R4, Cutler DF6.
Developmental cell.Dev Cell.2014 May 12;29(3):292-304. doi: 10.1016/j.devcel.2014.03.021. Epub 2014 May 1.
Weibel-Palade bodies (WPBs), endothelial-specific secretory granules that are central to primary hemostasis and inflammation, occur in dimensions ranging between 0.5 and 5 μm. How their size is determined and whether it has a functional relevance are at present unknown. Here, we provide evidence fo
PMID 24794632

Japanese Journal

Recognition of bacterial compounds by aortic endothelial cells activates Weibel-Palade body exocytosis

Into Takeshi,Matsushita Kenji
炎症・再生 : 日本炎症・再生医学会雑誌 = Inflammation and regeneration 27(2), 112-116, 2007-03-25
NAID 10019482581

Pathogen recognition by Toll-like receptor 2 activates Weibel-Palade body exocytosis in human aortic endothelial cells

INTO T
The Journal of biological chemistry 282, 8134-8141, 2007
NAID 80018644960

Angiogenesis of glioma : evaluation of ultrastructural characteristics of microvessels and tubular bodies (Weibel-Palade) in endothelial cells and immunohistochemical findings with VEGF and p53 protein

MIYAGAMI Mitsusuke,KATAYAMA Yoichi
Medical molecular morphology : official journal of the Japanese Society for Clinical Molecular Morphology 38(1), 36-42, 2005-03-01
NAID 10018374465

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英: Weibel-Palade body
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[1] Weibel ER, Palade GE; Palade (October 1964). "New cytoplasmic components in arterial endothelia". J. Cell Biol. 23 (1): 101–12. doi:10.1083/jcb.23.1.101. PMC 2106503. PMID 14228505.

[2] Wagner, D. D.; Olmsted, J. B.; Marder, V. J. (October 1982). "Immunolocalization of von Willebrand protein in Weibel–Palade bodies of human endothelial cells" (PDF). J. Cell Biol. 95 (1): 355–60. doi:10.1083/jcb.95.1.355. PMC 2112360. PMID 6754744.

[3] Tuma, Ronald F.; Durán, Walter N.; Ley, Klaus, eds. (2008). Microcirculation (2nd ed.). Amsterdam: Elsevier/Academic Press. p. 38. ISBN 978-0-12-374530-9.

[4] Bonfanti, R.; Furie, B. C.; Furie, B.; Wagner, D. D. (1 April 1989). "PADGEM (GMP140) is a component of Weibel–Palade bodies of human endothelial cells" (PDF). Blood 73 (5): 1109–12. PMID 2467701.

[5] McEver, R. P.; Beckstead, J. H.; Moore, K. L.; Marshall-Carlson, L.; Bainton, D. F. (July 1989). "GMP-140, a platelet alpha-granule membrane protein, is also synthesized by vascular endothelial cells and is localized in Weibel–Palade bodies". J. Clin. Invest. 84 (1): 92–9. doi:10.1172/JCI114175. PMC 303957. PMID 2472431.

[doyle-6] Doyle, E. L.; Ridger, V.; Ferraro, F.; Turmaine, M.; Saftig, P.; Cutler, D. F. (Oct 13, 2011). "CD63 is an essential cofactor to leukocyte recruitment by endothelial P-selectin". Blood 118 (15): 4265–73. doi:10.1182/blood-2010-11-321489. PMID 21803846.

[valentijn-7] ^ ^a ^b ^c ^d ^e ^f ^g Valentijn KM, Sadler JE, Valentijn JA, Voorberg J, Eikenboom J (May 12, 2011). "Functional architecture of Weibel-Palade bodies". Blood 117 (19): 5033–5043. doi:10.1182/blood-2010-09-267492. PMID 21266719.

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案内

Weibel-Palade body