Absence of Goodpasture's antigen in male patients with familial nephritis.
Absence of Goodpasture's antigen in male patients with familial nephritis.
复制标题
患有家族性肾炎的男性患者缺乏古德帕斯彻抗原。
DOI：
10.1016/s0272-6386(83)80043-2
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
Michael,AF
中科院分区：
文献类型：
--
作者：
Jeraj,K;Kim,Y;Vernier,RL;Fish,AJ;Michael,AF
用间接免疫荧光法测定了5种家族性肾炎9例患者肾小球基底膜（GBM）中GP抗原的存在。在7名男性中未检测到GP抗原，但在受感染的姐妹和母亲，未受影响的兄弟和13名正常对照中存在GP抗原。单克隆抗体鉴定的其他GBM抗原的持久性支持了这一发现在受影响男性中的特异性。在受影响的男性中缺乏GP抗原，并在相关的女性中持续存在，这表明可能存在x连锁显性遗传模式。我们认为GP抗原的缺失导致男性的严重疾病，而其在相关女性中的存在与轻度疾病相关。
The presence of Goodpasture's (GP) antigen in the glomerular basement membrane (GBM) of the kidney was evaluated by indirect immunofluorescence in nine patients with familial nephritis from five kindreds. The GP antigen was not detected in seven males but was present in an affected sister and mother, an unaffected brother, and 13 normal controls. The specificity of this finding in affected males is supported by the persistence of other GBM antigens identified by monoclonal antibodies. The lack of GP antigen in affected males and its persistence in related females with the disease suggests a possible X-linked dominant mode of inheritance. We propose that the absence of GP antigen leads to severe disease in the male, whereas its presence in related females is associated with mild disease.