IMMUNOHISTOCHEMICAL STUDY OF ALPHA-1-5 CHAINS OF TYPE-IV COLLAGEN IN HEREDITARY NEPHRITIS
IMMUNOHISTOCHEMICAL STUDY OF ALPHA-1-5 CHAINS OF TYPE-IV COLLAGEN IN HEREDITARY NEPHRITIS
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DOI：
10.1038/ki.1994.413
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发表时间：
1994-11-01
影响因子：
19.6
通讯作者：
SADO, Y
中科院分区：
文献类型：
--
作者：
NAKANISHI, K;YOSHIKAWA, N;SADO, Y
用间接免疫荧光法检测了23例遗传性肾炎家系肾小球基底膜(GBM)和表皮基底膜(EBM)中IV型胶原α1-5链[α1-5(IV)]分布。将这些家系分为三个临床病理组。第一组(10个家系)患者表现为广泛的基底膜“编织”型，并有肾炎家族史。第二组(6个家系)患者无肾炎家族史，表现为广泛的“编织”改变。组III(7个家系)患者表现为广泛的肾小球基底膜减弱，但无“编织”改变，并有肾炎和慢性肾功能衰竭的家族史。阿尔法1(IV)和阿尔法2(IV)在所有受影响和未受影响的家庭成员和对照中都存在。所有正常家系成员和对照在基底膜表达α3(IV)、α4(IV)和α5(IV)，在EBM以弥漫性方式表达α5(IV)。所有I组家系和3个II组家系在男性患者中均表现为GBM和EBM中的α5(IV)抗原完全丢失，而在女性患者中表现为节段性丢失。在这些家系中，重度肾炎男性患者的肾小球基底膜中α3(IV)和α4(IV)抗原完全消失，而轻度肾炎男性患者的肾小球基底膜中存在α3(IV)和α4(IV)。3个II组家系和所有III组家系表达Alpha3-5(IV)抗原的方式与正常对照组相同。这些结果表明遗传性肾炎的异质性反映了多种α3-5(IV)的异常表达模式，EBM中α5(IV)的免疫组织化学检测是诊断X连锁Alport综合征的有用方法。
The distribution of alpha 1-5 chains of type IV collagen [alpha 1-5(IV)] in the glomerular basement membrane (GBM) and epidermal basement membrane (EBM) of 23 families with hereditary nephritis was examined by indirect immunofluorescence. These families were divided into three clinicopathological groups. Group I (10 families) patients showed a widespread ''basket weave'' pattern of the GBM and a family history of nephritis was present. Group II (6 families) patients showed a widespread ''basket weave'' change without a family history of nephritis. Group III (7 families) patients showed a widespread attenuation of the GBM but no ''basket weave'' change, and had a family history of nephritis and chronic renal failure. alpha 1(IV) and alpha 2(IV) were present in all affected and unaffected family members and controls. All normal family members and controls expressed alpha 3(IV), alpha 4(IV) and alpha 5(IV) in the GBM and alpha 5(IV) in the EBM in a diffuse pattern. All group I families and three of the group II families exhibited complete loss of the alpha 5(IV) antigen from the GBM and EBM in male patients, and segmental loss of the alpha 5(IV) antigen in female patients. In these families the (alpha 3(IV) and alpha 4(IV) antigens were completely lost from the GBM in male patients with severe nephritis, whereas alpha 3(IV) and alpha 4(IV) were present but diminished in male patients with mild nephritis. Three group II and all group III families expressed the alpha 3-5(IV) antigens in an identical manner to that of normal controls. These findings indicate that the heterogeneity of hereditary nephritis reflects a variety of aberrant expression patterns of alpha 3-5(IV) and that immunohistochemical examination of alpha 5(IV) in the EBM is a useful method for the diagnosis of X-linked Alport syndrome.