MBL deficiency
| MBL deficiency | |
|---|---|
| Other names | Mannose-binding lectin deficiency |
 | |
| Structure of the MBL2 protein. | |
| Specialty | Immunology |
MBL deficiencyormannose-binding lectin deficiencyis an illness that has an impact on immunity. Low levels ofmannose-binding lectin,animmune systemprotein, are present in the blood of those who have this illness. It's unclear if this deficiency increases the risk of recurrent infections in those who are affected.[1]
Signs and symptoms
[edit]Specific infection susceptibility has been linked to low MBL levels.[2]This is especially true when it comes to chronic illnesses likecystic fibrosis,chemotherapy-induced neutropenia,and weakened immunity, which are all present in the neonatal period.[3]Moreover,septic shockand death have been connected to MBL deficiency.[4]
Causes
[edit]Mannose-binding lectin deficiency can result from mutations in theMBL2gene.[1]
Mechanism
[edit]Mannose-binding lectin(MBL) is a solubleinnate immune systempattern recognition molecule.[5]MBL activates the complementlectin pathwayby binding toglycoconjugateson the surface of a variety of clinically significant bacteria, viruses, and fungi that containmannose,fucose,orN-acetylglucosamine.[6][7]MBL stimulatesleucocytechemotaxisand activation as well asphagocytosisthroughcomplement activation.[8][9]Additionally, MBL may contribute to the resolution of lung inflammation byapoptosiscell clearance and proinflammatorycytokinesecretion suppression.[10][11]
Epidemiology
[edit]Depending on the definition of deficiency, MBL deficiency affects 10–30% of individuals, making it one of the most prevalent immune defects.[12]
References
[edit]- ^ab"Mannose-binding lectin deficiency: MedlinePlus Genetics".MedlinePlus.May 1, 2018.RetrievedJanuary 22,2024.
- ^Haibrich, Michelle; Ben-Shoshan, Moshe; Mccusker, Christine (2012)."Mannose Binding Lectin Deficiency: More than Meets the Eye".Clinical Medicine Insights: Pediatrics.6.SAGE Publications: 89–94.doi:10.4137/cmped.s9860.ISSN1179-5565.PMC3620812.PMID23641170.
- ^Eddie Ip, W. K.; Takahashi, Kazue; Alan Ezekowitz, R.; Stuart, Lynda M. (June 29, 2009). "Mannose-binding lectin and innate immunity".Immunological Reviews.230(1). Wiley: 9–21.doi:10.1111/j.1600-065x.2009.00789.x.ISSN0105-2896.PMID19594626.S2CID44299051.
- ^Dommett, R. M.; Klein, N; Turner, M. W. (2006)."Mannose-binding lectin in innate immunity: past, present and future".Tissue Antigens.68(3). Wiley: 193–209.doi:10.1111/j.1399-0039.2006.00649.x.ISSN0001-2815.PMC7169806.
- ^Kilpatrick, D (September 19, 2002). "Mannan-binding lectin: clinical significance and applications".Biochimica et Biophysica Acta (BBA) - General Subjects.1572(2–3). Elsevier BV: 401–413.doi:10.1016/s0304-4165(02)00321-5.ISSN0304-4165.PMID12223282.
- ^Neth, Olaf; Jack, Dominic L.; Dodds, Alister W.; Holzel, Helen; Klein, Nigel J.; Turner, Malcolm W. (2000)."Mannose-Binding Lectin Binds to a Range of Clinically Relevant Microorganisms and Promotes Complement Deposition".Infection and Immunity.68(2). American Society for Microbiology: 688–693.doi:10.1128/iai.68.2.688-693.2000.ISSN0019-9567.PMC97193.PMID10639434.
- ^Møller-Kristensen, Mette; Ip, W. K. Eddie; Shi, Lei; Gowda, Lakshmi D.; Hamblin, Michael R.; Thiel, Steffen; Jensenius, Jens Chr.; Ezekowitz, R. Alan B.; Takahashi, Kazue (February 1, 2006)."Deficiency of Mannose-Binding Lectin Greatly Increases Susceptibility to Postburn Infection withPseudomonas aeruginosa".The Journal of Immunology.176(3). The American Association of Immunologists: 1769–1775.doi:10.4049/jimmunol.176.3.1769.ISSN0022-1767.PMC3071691.PMID16424207.
- ^Thiel, Steffen; Vorup-Jensen, Thomas; Stover, Cordula M.; Schwaeble, Wilhelm; Laursen, Steen B.; Poulsen, Knud; Willis, Anthony C.; Eggleton, Paul; Hansen, Søren; Holmskov, Uffe; Reid, Kenneth B. M.; Jensenius, Jens C. (1997). "A second serine protease associated with mannan-binding lectin that activates complement".Nature.386(6624). Springer Science and Business Media LLC: 506–510.Bibcode:1997Natur.386..506T.doi:10.1038/386506a0.ISSN0028-0836.PMID9087411.
- ^Wallis, Russell (2007). "Interactions between mannose-binding lectin and MASPs during complement activation by the lectin pathway".Immunobiology.212(4–5). Elsevier BV: 289–299.doi:10.1016/j.imbio.2006.11.004.hdl:2381/15008.ISSN0171-2985.PMID17544814.
- ^Hodge, Sandra; Matthews, Geoffrey; Dean, Melinda M.; Ahern, Jessica; Djukic, Michael; Hodge, Greg; Jersmann, Hubertus; Holmes, Mark; Reynolds, Paul N. (2010). "Therapeutic Role for Mannose-Binding Lectin in Cigarette Smoke–Induced Lung Inflammation?".American Journal of Respiratory Cell and Molecular Biology.42(2). American Thoracic Society: 235–242.doi:10.1165/rcmb.2008-0486oc.ISSN1044-1549.PMID19411612.
- ^Wang, Mingyong; Chen, Yue; Zhang, Yani; Zhang, Liyun; Lu, Xiao; Chen, Zhengliang (March 7, 2011)."Mannan-binding lectin directly interacts with Toll-like receptor 4 and suppresses lipopolysaccharide-induced inflammatory cytokine secretion from THP-1 cells".Cellular & Molecular Immunology.8(3). Springer Science and Business Media LLC: 265–275.doi:10.1038/cmi.2011.1.ISSN1672-7681.PMC4012877.PMID21383675.
- ^Eisen, Damon P. (July 7, 2009)."Mannose-Binding Lectin Deficiency and Respiratory Tract Infection".Journal of Innate Immunity.2(2). S. Karger AG: 114–122.doi:10.1159/000228159.ISSN1662-811X.PMC7179718.PMID20375630.
Further reading
[edit]- Thiel, S.; Frederiksen, P.D.; Jensenius, J.C. (2006)."Clinical manifestations of mannan-binding lectin deficiency".Molecular Immunology.43(1–2). Elsevier BV: 86–96.doi:10.1016/j.molimm.2005.06.018.ISSN0161-5890.PMC7132399.PMID16023210.
- Chalmers, James D; McHugh, Brian J; Doherty, Catherine; Smith, Maeve P; Govan, John R; Kilpatrick, David C; Hill, Adam T (2013). "Mannose-binding lectin deficiency and disease severity in non-cystic fibrosis bronchiectasis: a prospective study".The Lancet Respiratory Medicine.1(3). Elsevier BV: 224–232.doi:10.1016/s2213-2600(13)70001-8.hdl:20.500.11820/dd3e4829-51fd-4bbc-82cf-66f40a88dbab.ISSN2213-2600.