Von Willebrand factor
| Von Willebrand factor | ||
|---|---|---|
|
Existing structural data : 1ao3 , 1atz , 1auq , 1fe8 , 1fns , 1ijb , 1ijk , 1m10 , 1oak , 1sq0 , 1u0n , 1uex , 2adf |
||
| Properties of human protein | ||
| Mass / length primary structure | 225.7 kDa / 2050 AS | |
| Secondary to quaternary structure | Multimer | |
| Precursor | (2813 AS; 309 kDa) | |
| Identifier | ||
| Gene names | vWF ; F8VWF | |
| External IDs | ||
| Occurrence | ||
| Parent taxon | Euteleostomi | |
| Orthologue | ||
| human | mouse | |
| Entrez | 7450 | 22371 |
| Ensemble | ENSG00000110799 | ENSMUSG00000001930 |
| UniProt | P04275 | Q2I0J7 |
| Refseq (mRNA) | NM_000552 | NM_011708 |
| Refseq (protein) | NP_000543 | NP_035838 |
| Gene locus | Chr 12: 5.93 - 6.1 Mb | Chr 6: 125.51 - 125.65 Mb |
| PubMed search | 7450 |
22371
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The Von Willebrand Factor (VWF) is a protein , which as carrier protein of blood coagulation factor VIII an important role in hemostasis plays and after Erik Adolf von Willebrand (1870-1949), a Finnish internist , was named.
Von Willebrand factor is produced by both megakaryocytes and the endothelial cells that make up the inner wall of a blood vessel (the intima ). In the blood platelets (thrombocytes) as constrictions of the megakaryocytes, it is stored in the α-granules; the vascular endothelium transfers it to the subendothelial matrix for storage. If the inner wall of the endothelium ruptures, the underlying proteins of the vessel wall, including collagens , are exposed . The Von Willebrand factor can bind to this. Certain cellular elements of the blood, the platelets ( thrombocytes ), have a docking point on their surface to which the Von Willebrand factor can bind. This is called the Von Willebrand receptor or glycoprotein Ib / V / IX. The Von Willebrand factor thus creates a bridge between the platelets and the injured vessel wall. The vWF can bind to already adherent platelets by means of glycoprotein IIb or IIIa.
The Von Willebrand factor thus has a direct effect on cellular hemostasis. It is not directly involved in plasmatic blood coagulation, as it is not required for the formation of fibrin. Due to its function as a carrier and protective protein for coagulation factor VIII, a deficiency in Von Willebrand factor or a defect in its protein structure due to this direct interaction nevertheless leads to an impairment of the plasmatic hemostasis (see Willebrand-Jürgens syndrome and Von Willebrand Disease ).
The concentration of the Von Willebrand factor depends on the blood group ; For example, people with blood group 0 measured 25-35% lower values than with blood groups A, B and AB. This is possibly one of the reasons for the partially varying prevalence of cardiovascular diseases between the blood groups. The Association of German Cardiologists attributes up to six percent of all heart diseases to unfavorable blood groups.
See also
literature
- EA von Willebrand: Hereditary pseudohemofili. In: Fin Läkaresällsk Handl. 68, 1926, pp. 87-112. (Reproduced in EA von Willebrand: Hereditary pseudohaemophilia. In: Haemophilia. 5 (3), 1999, pp. 223-231; discussion p. 222. doi: 10.1046 / j.1365-2516.1999.00302.x . PMID 10444294 )
- JE Sadler: Biochemistry and genetics of von Willebrand factor. In: Annu. Rev. Biochem. 67, 1998, pp. 395-424. doi: 10.1146 / annurev.biochem.67.1.395 . PMID 9759493 .
- AJ Jakobi, A. Mashaghi, SJ Tans, EG Huizinga: Calcium modulates force sensing by the von Willebrand factor A2 domain. In: Nature Commun. 2, 2011 Jul 12, p. 385.
- GG Levy, DG Motto, D. Ginsburg: ADAMTS13 turns 3. In: Blood. 106 (1), 2005, pp. 11-17. doi: 10.1182 / blood-2004-10-4097 . PMID 15774620 .
- JE Sadler, U. Budde, JC Eikenboom et al .: Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. In: J. Thromb. Haemost. 4 (10), 2006, pp. 2103-2114. doi: 10.1111 / j.1538-7836.2006.02146.x . PMID 16889557 .
- A. Vincentelli, S. Susen, T. Le Tourneau and others: Acquired von Willebrand syndrome in aortic stenosis. In: N. Engl. J. Med. 349 (4), 2003, pp. 343-349. doi: 10.1056 / NEJMoa022831 . PMID 12878741 .
- JL Moake: von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura. In: Semin. Hematol. 41 (1), 2004, pp. 4-14. doi: 10.1053 / j.seminhematol.2003.10.003 . PMID 14727254 .
- T. Bongers, M. de Maat, M. van Goor et al .: High von Willebrand factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability. In: Stroke. 37 (11), 2006, pp. 2672-2677. doi: 10.1161 / 01.STR.0000244767.39962.f7 . PMID 16990571 .
- FI Pareti, Y. Fujimura, JA Dent, LZ Holland, TS Zimmerman, ZM Ruggeri: Isolation and characterization of a collagen binding domain in human von Willebrand factor. In: J. Biol. Chem. 261 (32), 1986, pp. 15310-15315. PMID 3490481
Individual evidence
- ↑ https://www.info-von-willebrand.de/info-von-willebrand/wissen-und-therapie/diagnose.php
- ↑ http://flexikon.doccheck.com/de/Von-Willebrand-Faktor
- ↑ Loretta Bruhns: Prevention: The blood group determines the risk of disease. In: welt.de . February 23, 2015, accessed October 7, 2018 .