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儿童甘露聚糖结合凝集素遗传缺陷 被引量:1

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摘要 甘露聚糖结合凝集素(MBL)是一种钙离子依赖的C型血清凝集素,它可以选择性识别结合病原体表面的末端含甘露聚糖、N-乙酰葡萄糖胺的糖基,通过凝集素通路激活补体,或直接作为调理素介导调理噬菌作用,也可直接与病毒结合,中和病毒并抑制其扩散。MBL在天然免疫中起着重要作用,尤其对免疫机能发育尚未成熟的婴幼儿其作用更为重要。MBL基因突变是儿童天然免疫缺陷的一个危险因子,有三种突变型:G54D,G57E/Q和 R52C。基因突变破坏了 MBL分子的多聚化组装,使其不稳定,易降解,血清中浓度下降,活性受损,患儿可表现出对感染的易感性增加,或伴发其他免疫缺陷病。对严重或频发感染的患儿进行MBL血清浓度的检测及其基因突变的筛查,有助于诊断和治疗。国外已报道用纯化正常人血浆MBL治疗MBL遗传缺陷,重建调理噬菌功能。
出处 《国外医学(儿科学分册)》 2001年第5期253-256,共4页 Foreign Medical Sciences(Section of Pediatrics)
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  • 1Thiel S, Vorup-Jensen T, Stover CM, et al. A second serine protease associated with mannan-binding lectin that activates complement[J]. Nature, 1997,386(6624):506 - 510.
  • 2Tabona P, Mellor A, Summerfield JA. Mannose binding protein is involved in first-line host defence:evidence from transgenic mice[J].Immunology, 1995,85(1):153-159.
  • 3Garred P, Madsen HO, Hofmann B, et al. Increased frequency of homozygosity of abnormal mannan-binding-protein alleles in patients with suspected immunodeficiency[J]. Lancet, 1995, 346 (8980):941 - 943.
  • 4Summerfield JA, Sumiya M, Levin M, et al. Association of mutations in mannose binding protein gene with childhood infection in consecutive hospital series[J]. BMJ, 1997,314(7089):1229-1232.
  • 5Aittoniemi J, Baer M, Soppi E, et al. Mannan binding lectin deficiency and concomitant immunodefects[J]. Arch Dis Child,1998,78(3):245 - 248.
  • 6Koch A, Melbye M, Sorensen P, et al. Acute respiratory tract infections and mannose-binding lectin insufficiency during early childhood[J]. JAMA, 2001,285(10):1316 - 1321.
  • 7Kawasaki T. Structure and biology of mannan-binding protein,MBP, an important component of innate immunity[J]. Biochim Biophys Acta, 1999,1473(1):186 - 195.
  • 8Day AJ. The C-type carbohydrate recognition domain(CRD) superfamily[J]. Biochem Soc Trans, 1994,22(1):83-88.
  • 9Drickamer K. Ca2+ -dependent sugar recognition by animal lectins[J]. Biochem Soc Trans, 1996,24(1):146-150.
  • 10Neth O, Jack DL, Dodds AW, et al. Mannose-binding lectin binds to a range of clinically relevant microorganisms and promotes complement deposition[J]. Infect Immun, 2000,68(2):688 - 693.

同被引文献7

  • 1Kilpatrick DC. Mannan-binding leetin and its role in innate immunity[J]. Transfus Med,2002,12:335.
  • 2Turner MW, Hamvas RMJ. Mannose-binding lectin: structure, function, genetics and disease associations[J]. Rev Immunogenet, 2000.2 :305.
  • 3Kawai T, Suzuki Y, Eda S, et al. Molecular and biological characterization of rabbit mannan-binding protein (MBP)[ J ]. Glycobiology.1998,8:237.
  • 4Hansen S, Thiel S, Willis A, et al. Purification and characterization of two mannan-binding lectins from mouse serum[J]. J Immunol,2000,164:2610.
  • 5Storgaard P, Holm-Nielsen E, Andersen O, et al. Isolation and characterization of porcine mannan-binding proteins of different size and uhrastructure[ J ]. Scand J Immunol, 1996,43 : 289.
  • 6Holmskov U, Holt P, Raid KBM,et al. Purification and characterization of bovine mannan-binding protein[J]. Glycobiology, 1993,3 : 147.
  • 7陈政良,韩强涛,易正山,张丽芸,卢晓,彭良平.人血浆MBL的分离纯化及其特性鉴定[J].免疫学杂志,1998,14(1):12-14. 被引量:17

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