Mannose Binding Lectin Deficiency and Clinical Features

Year 2013, Volume: 22 Issue: 4, 565 - 574, 01.12.2013

Ertuğrul Erken

Abstract

Innate immunity consists of macrophages, neutrophils, natural killer cells, mucosal immunuglobulins and the comlement system. Mannose binding lectin (MBL) takes part in innate immunity through opsonisation and complement activation. MBL deficiency is associated with some infections and autoimmune disorders. However some studies indicate that MBL deficiency alone is not essential for immunity but it may intensify the clinic picture of an immune deficiency that already exists. This article refers to clincal studies related to MBL and brings up the clinical importance of MBL deficiency.

Keywords

Mannose binding lectin, lectin pathway, mannose binding lectin deficiency.

Mannoz Bağlayıcı Lektin Eksikliği ve Klinik Bulgular

Abstract

Doğal immünite makrofajlar, doağal öldürücü hücreler, salgısal immünglobulinler ve kompleman
sistemi gibi bileşenlerden oluşur. Mannoz bağlayıcı lektin ise opsonizasyon ve kompleman aktivasyonu
aracılığı ile doğal immuniteye katkıda bulunmaktadır. Mannoz bağlayıcı lektin eksikliği bazı enfeksiyöz
ve otoimmün hastalıklarla ilişkili bulunmuştur. Ancak bazı araştırmalar Mannoz bağlayıcı lektin
eksikliğinin doğal immünite için tek başına önemli olmadığını ancak halihazırda mevcut olan bir
immün yetersizliğin klinik bozukluklarını artırabileceğini öngörmektedir. Bu yazıda mannoz bağlayıcı
lektin ile ilgili klinik çalışmalara ve mannoz bağlayıcı lektin eksikliğinin klinik önemi ele alınmıştır.

Keywords

Mannoz bağlayıcı lektin, lektin yolağı, mannoz bağlayıcı lektin eksikliği.

References

• Degn SE, Thiel S, Jensenius JC. New perspectives on mannan-binding lectin-mediated complement activation. Immunobiology. 2007; 212:301-11.
• Worthley DL, Bardy PG, Mullighan CG. Mannose binding lectin biology and clinical implications. Intern Med J. 2005; 35:548-55.
• Neth O, Jack DL, Dodds AW, Holzel H, Klein NJ, Turner MW. Mannose-binding lectin binds to a range of clinically relevant microorganisms and promotes complement deposition. Infect Immun. 2000; 68: 688-93.
• Stuart LM, Takahashi K, Shi L, Savill J, Ezekowitz RA. Mannose binding lectin deficient mice display defective apoptotic cell clearance but no autoimmune phenotype. J Immunol. 2005; 174:3220-6.
• Soell M, Lett E, Holveck F, Schöller M, Wachsmann D, Klein JP. Activation of human monocytes by streptococcal rhamnose glucose polymers is mediated by CD14 antigen, and mannan binding protein inhibits TNF-alpha release. J Immunol. 1995; 154:851-60.
• Atkinson JP. Complement System. In Kelley’s Textbook of Rheumatology, 7th ed. (Eds ED Haris, RC Budd, GS Firestein, MC Genovese, JS Sergent, S Ruddy): 342-55. USA, Elsevier Saunders, 2005. Madsen HO, Garred P, Thiel S, Kurtzhals JA, Lamm LU, Ryder LP et al. Interplay between promoter and structural gene variants control basal serum level of mannan-binding protein. J Immunol. 1995; 155:3013-20.
• Garred P, Larsen F, Seyfarth J, Fujita R, Madsen HO. Mannose-binding lectin and its genetic variants. Genes Immun. 2006; 7:85-94.
• Lipscombe RJ, Sumiya M, Hill AV, Lau YL, Levinsky RJ, Summerfield JA et al. High frequencies in African and non-African populations of independent mutations in the mannose binding protein gene. Hum Mol Genet. 1992; 1: 709-15.
• Thiel S, Frederiksen PD, Jensenius JC. Clinical manifestations of mannan-binding lectin deficiency. Mol Immunol. 2006; 43:86-96.
• Garcia-Laorden MI, Sole-Violan J, Rodriguez de Castro F, Aspa J, Briones ML, Garcia-Saavedra A et al. Mannose-binding lectin and mannose-binding lectin-associated serine protease 2 in susceptibility, severity, and outcome of pneumonia in adults. J Allergy Clin Immunol. 2008; 122:368-74.