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Animal Models of Inherited Complement Deficiency

机译：遗传性补体缺乏症的动物模型

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The initial description of murine strains deficient in complement component CS has been followed by the recognition in a range of animal species of a variety of natural complement component deficiencies, many of which have been characterized at the molecular level. The use of such species in inflammatory and infectious experimental models has led to significant progress in understanding the role of specific complement factors (and pathways) in disease pathogenesis. Deficiencies of early complement factors are characterized by impairment of immune response, possibly due to defective processing of immune complexes. Complete (but not partial) deficiency of the central component C3 predisposes affected animals to significant risk of infec- tion and renal disease. Studies in species deficient in the terminal pathway component C6 are particularly relevant for investigating the pathogenetic role of the terminal membrane attack complex (MAC), implicat- ing it as a causative agent in diverse inflammatory insults such as reperfusion injury, glomerular damage, and xenograft hyperacute rejection. Further investigations in such naturally deficient strains, added to results derived from studies in knockout animals.. are likely to expand our understanding of the role of the activated complement system in experimental inflammatory disease, with significant potential implications for the treatment of human disease.
机译：在缺乏补体成分CS的鼠类菌株的最初描述之后，在一系列动物物种中认识到各种天然补体成分缺陷，其中许多已在分子水平上表征。在炎症和感染性实验模型中使用此类物种已导致在理解特定补体因子（和途径）在疾病发病机理中的作用方面取得了重大进展。早期补体因子的缺乏症的特征是免疫应答受损，这可能是由于免疫复合物的加工缺陷所致。中央成分C3的完全（而非部分）缺乏使患病动物容易患感染和肾脏疾病。缺乏终端途径组分C6的物种的研究与研究终端膜攻击复合物（MAC）的致病作用特别相关，这暗示它是多种炎症损伤（例如再灌注损伤，肾小球损伤和异种移植）的病原体。超急性排斥反应。对这种自然缺乏菌株的进一步研究，加上对基因敲除动物研究的结果，可能会扩大我们对活化补体系统在实验性炎性疾病中的作用的理解，对人类疾病的治疗具有重要的潜在意义。

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