C3 dysregulation due to factor H deficiency is mannan-binding lectin-associated serine proteases (MASP)-1 and MASP-3 independent in vivo.
C3 dysregulation due to factor H deficiency is mannan-binding lectin-associated serine proteases (MASP)-1 and MASP-3 independent in vivo.
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DOI：
10.1111/cei.12244
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发表时间：
2014-04
影响因子：
4.6
通讯作者：
Pickering MC
中科院分区：
文献类型：
--
作者：
Ruseva MM;Takahashi M;Fujita T;Pickering MC
补体替代途径不受控制的激活与补体介导的肾脏疾病有关。 B 因子和 D 因子是该途径的重要组成部分，而 H 因子 (FH) 是其主要调节因子。在完全 FH 缺乏症中，通过旁路途径不受控制的 C3 激活会导致血浆 C3 耗竭和补体介导的肾脏疾病。这些依赖于因子 B。甘露聚糖结合凝集素相关丝氨酸蛋白酶 1 和 3 (MASP-1、MASP-3) 最近已被证明可通过将前因子 D 裂解为其活性形式因子 D 来促进旁路途径激活。我们研究了 MASP-1 和 MASP-3 对实验性完全 FH 缺陷中不受控制的旁路途径激活的贡献。 FH 和 MASP-1/MASP-3 的共同缺陷并不能改善 FH 缺陷小鼠的血浆 C3 激活或肾小球 C3 积累。我们的数据表明，MASP-1 和 MASP-3 对于完全 FH 缺陷的替代途径激活不是必需的。
Uncontrolled activation of the complement alternative pathway is associated with complement-mediated renal disease. Factor B and factor D are essential components of this pathway, while factor H (FH) is its major regulator. In complete FH deficiency, uncontrolled C3 activation through the alternative pathway results in plasma C3 depletion and complement-mediated renal disease. These are dependent on factor B. Mannan-binding lectin-associated serine proteases 1 and 3 (MASP-1, MASP-3) have been shown recently to contribute to alternative pathway activation by cleaving pro-factor D to its active form, factor D. We studied the contribution of MASP-1 and MASP-3 to uncontrolled alternative pathway activation in experimental complete FH deficiency. Co-deficiency of FH and MASP-1/MASP-3 did not ameliorate either the plasma C3 activation or glomerular C3 accumulation in FH-deficient mice. Our data indicate that MASP-1 and MASP-3 are not essential for alternative pathway activation in complete FH deficiency.
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影响因子：
11.4
作者：
MORLEY, BJ;CAMPBELL, RD
通讯作者：
CAMPBELL, RD
影响因子：
4.4
作者：
Iwaki, Daisuke;Kanno, Kazuko;Fujita, Teizo
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Fujita, Teizo
影响因子：
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Arend, William P.
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作者：
Fakhouri, Fadi;Fremeaux-Bacchi, Veronique;Pickering, Matthew C.
通讯作者：
Pickering, Matthew C.
影响因子：
15.3
作者：
LESAVRE, PH;MULLEREBERHARD, HJ
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MULLEREBERHARD, HJ