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目的观察Alport综合征(AS)患者肾组织层粘连蛋白α2链、α5链和γ1链的分布。方法采用免疫荧光方法,运用普通荧光和激光共聚焦扫描显微镜观察抗层粘连蛋白α2链、α5链和γ1链单克隆抗体在肾组织中的沉积情况。肾组织标本来自11例AS患者,其中男8例,女3例,年龄11～52岁。10例患者符合X伴性显性遗传(XLAS),1例女性患者符合显性遗传。8例男性XLAS患者肾小球基底膜(GBM)、远端肾小管基底膜均无Ⅳ型胶原α3、5链沉积,表皮基底膜(EBM)无α5(Ⅳ)链沉积;2例女性XLAS患者肾组织α3、5(Ⅳ)链和EBMα5(Ⅳ)链均呈不连续表达,另1例女性患者则同正常肾组织。正常人肾组织标本作为正常对照,9例IgA肾病(IgAN)、6例局灶节段硬化性肾小球病(FSGS)、5例薄基膜病(TBMD)和6例肾小球轻微病变(GML)患者作为疾病对照。结果正常人肾组织层粘连蛋白α2链主要沉积于肾小球系膜区,层连蛋白α5、γ1链沉积于GBM、所有肾小管基底膜和入球小动脉基底膜。10例XLAS和1例显性遗传AS患者肾组织除了肾小球系膜区外,层粘连蛋白α2链在GBM上亦出现表达。IgAN、TBMD和FSGS患者层粘连蛋白α2链仅在肾小球系膜区沉积。层粘连蛋白α5、γ1链在AS患者和其他肾脏病组织沉积同正常肾组织。结论层粘连蛋白α2链在AS患者GBM出现异位表达,为继Ⅳ型胶原α链异常之后发现的又-AS基底膜成分的异常,其对于AS可能具有重要的诊断价值。
Objective To observe the distribution of laminin α2 chain, α5 chain and γ1 chain in renal tissues of patients with Alport syndrome (AS). Methods Immunofluorescence staining was used to observe the deposition of anti-laminin α2 chain, α5 chain and γ1 chain monoclonal antibody in renal tissues by using ordinary fluorescence and laser scanning confocal microscope. Renal tissue samples from 11 cases of AS patients, including 8 males and 3 females, aged 11 to 52 years. Ten patients were eligible for X-linked dominant inheritance (XLAS), and one female patient was in line with dominant inheritance. There was no deposition of α3,5 chain of collagen Ⅳ in the glomerular basement membrane (GBM) and distal tubule basement membrane in 8 male XLAS patients, no deposition of α5 (Ⅳ) chain in the epidermal basement membrane (EBM); 2 female XLAS patients Renal α3,5 (Ⅳ) chain and EBMα5 (Ⅳ) chain were discontinuous expression of the other cases of female patients with normal renal tissue. Nine normal IgA nephropathy (IgAN), six focal segmental glomerulonephritis (FSGS), five thin basement membrane disease (TBMD) and six mild glomerular lesions (GML) patients as disease control. Results Normal human renal laminin α2 chain mainly deposited in the glomerular mesangial area, laminin α5, γ1 chain deposited in GBM, all of the tubular basement membrane and the basilar artery into the arterioles. In addition to the glomerular mesangial area, laminin α2 chain was also expressed on GBM in 10 cases of XLAS and 1 case of dominant genetic AS. The laminin α2 chain of IgAN, TBMD and FSGS patients only deposits in the mesangial area. Laminin α5, γ1 chain in AS patients and other nephritic tissue deposition with normal renal tissue. Conclusion Laminin α2 chain is ectopically expressed in GBM in patients with AS, which may be an important diagnostic value for AS after the aberrant α-chain abnormality of type Ⅳ collagen.