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Arfgengur skortur í ræsisameindum lektínferils komplímentvirkjunar

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Authors
Helga Bjarnadóttir
Björn Rúnar Lúðvíksson
Issue Date
2010-11-01

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Other Titles
Inherited deficiency of the initiator molecules of the lectin-complement pathway
Citation
Læknablaðið 2010, 96(10):611-7
Abstract
The complement system is an important immune system. Its activation results in membranolytic elimination of microbes and opsonization. The classical, alternative and lectin pathways (LP) activate complement. Either mannan-binding lectin (MBL), ficolin-1, ficolin-2 or ficolin-3 initiate the LP through associated serine protease (MASP-2) after binding to microorganisms'surface carbohydrate patterns. Genetic polymorphisms behind MBL deficiency are rather common. Numerous studies indicate that MBL deficiency is a risk factor for invasive and recurrent infections, especially when other immune systems are immature, deficient or compromised. Research in ficolins is limited but last year ficolin-3 deficiency was described. This review focuses on these recently WHO defined immunodeficiencies.
Komplímentkerfið er mikilvæg ónæmisvörn.Virkjun þess leiðir til áthúðunar og himnurofs sýkla. Þrír ferlar virkja komplímentkerfið, klassíski, styttri og lektín. Lektínferillinn er ýmist ræstur af lektínunum mannanbindilektín (MBL), fíkólín-1, fíkólín-2 eða fíkólín-3 gegnum serínpróteasa (MASP-2). Lektínin hafa svipaða byggingu og bindast sykrumynstrum á yfirborði sýkla. Erfðabreytileiki í MBL2 geninu sem veldur skorti er frekar algengur. Fjöldi rannsókna hefur sýnt að skortur er áhættuþáttur fyrir ífarandi og endurteknar sýkingar, sérstaklega þar sem aðrar ónæmisvarnir eru óþroskaðar, bældar eða gallaðar. Rannsóknir á fíkólínum eru á styttra veg komnar, en á síðasta ári var fíkólín-3-skorti lýst. Í þessu yfirliti verður fjallað um þessa ónæmisgalla sem WHO hefur nýlega skilgreint.
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Neðst á síðunni er hægt að nálgast greinina í heild sinni með því að smella á hlekkinn Skoða/Opna(view/open)
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http://www.laeknabladid.is
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Icelandic Journal Articles (Peer Reviewed)

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