薄基底膜肾病患者基底膜Ⅳ型胶原α链的表达  

EXPRESSIONS OF TYPE Ⅳ COLLAGEN α CHAINS IN BASEMENT MEMBRANES OF THIN BASEMENT MEMBRANE NEPHRITIS

作　　者：丁洁[1] 柴青[1] 刘景城[1] 张英[1] 杨霁云[1] 

机构地区：[1]北京医科大学第一医院儿科,北京100034

出　　处：《肾脏病与透析肾移植杂志》2000年第2期123-125,共3页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金! (3 9770780)

摘　　要：目的 :检测薄基底膜肾病 (TBMN)患者基底膜中Ⅳ型胶原α链的表达 ,探讨其发病机制 ;并与Alport综合征 (AS)比较 ,为鉴别TBMN和AS提供帮助。　 方法 :用抗Ⅳ型胶原不同α链的单克隆抗体 ,采用链菌素亲生物素 过氧化酶连接法检测了 6例TBMN、3例X连锁型AS男性患儿及 2例正常对照肾脏组织基底膜中Ⅳ型胶原α1、α3、α4和α5链的表达 ;采用间接免疫荧光法检测了 3例TBMN、4例X链锁型AS男性患儿及 4例正常对照皮肤基底膜中α1(Ⅳ )和α5 (Ⅳ )链的表达。　 结果 :TBMN患儿肾小球基底膜中Ⅳ型胶原α1、α3～ 5链表达同正常 ,均为沿基底膜连续线状沉积。X连锁型AS男性患儿肾小球基底膜显示Ⅳ型胶原α1链染色阳性 ,而α3～ 5链染色均为阴性。TBMN、AS患儿及正常对照皮肤基底膜中α1(Ⅳ )链染色均为沿基底膜连续线状沉积 ;而α5 (Ⅳ )链染色仅在AS为阴性 ,在TBMN和正常对照均为连续线状沉积。　 结论 :TBMN患儿基底膜中α(Ⅳ )链表达不同于AS。OBJECTIVE To detect the basement membrane α(Ⅳ) chains expressions in thin basement membrane nephritis(TBMN)and Alport syndrome,and to find the difference of the expressions between the two diseases. METHODOLOGY The monoclonal antibodies against type Ⅳ collagen α chains were applied in the methods of streptavidin peroxidase conjugated staining(SP)and indirect immunofluorescence.The expressions of α(Ⅳ)chains in glomerular basement membrane(GBM) were detected by SP in 6 TBMN,2 X linked AS male patients,and 2 normal controls.The expressions of α(Ⅳ)chains in epidermal basement membrane(EBM)were detected by IF in 3 TBMN,4 X lined AS male patients and 4 normal controls. RESULTS The α1(Ⅳ) and α3 5(Ⅳ)chains expressions were found linear positive along the GBM in TBMN patients,in a same pattern as in the normal controls.In X linked AS male patients,the expression of α1(Ⅳ)chain was positive while the expressions of α3 5(Ⅳ)chains were negative in GBM.The expressions of α1(Ⅳ)chains were found positive in a linear pattern along EBM in TBMN patients,while the expression of α5(Ⅳ) chain in EBM was found negative in X linked AS male patients. CONCLUSION The expressions of α(Ⅳ) chains in EBM and GBM can be used in the differential diagnosis between TBMN and AS.

关 键 词：薄基底膜肾病 Ⅳ型胶原 ALPORT综合征 

分 类 号：R692[医药卫生—泌尿科学]