日本登特病患者各种尿蛋白的电泳分析

Kumiko Sawada, T. Matsuyama, T. Sekine, K. Shiba, Kenji Sato
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引用次数: 0

摘要

患者尿中α1-微球蛋白(α1-m)、β2-微球蛋白(β2-m)和视黄醇结合蛋白(RBP)的分泌量较高。结果发现,牙凹病患者血清α1-m、β2-m和RBP水平分别是正常人的20倍、130倍和200倍。这些结果证实了以前的研究结果。醋酸纤维素膜电泳(CAE)显示,Dent病患者尿蛋白组分表现为白蛋白前期、白蛋白值低、α1-G和α2-球蛋白(α2-G)值相等、β-球蛋白(β-G)消失、α2-G慢(RBP)和β2- g慢(β2-m)。采用十二烷基硫酸钠-聚丙烯酰胺凝胶电泳(SDS-PAGE)后的western blot方法,对凹痕病1 (CLCN 5突变)和凹痕病2 (OCRL 1突变)患者及健康人的尿白蛋白(ALB)、α1-m、β2-m和RBP进行了检测。除ALB主带(67 kDa)外,还检测到84 ~ 130 kDa的6个波段。除α1-m主带(34 kDa)外,共检测到51 ~ 92 kDa的7个条带。在β2-m主带(14 kDa)之外,还检测到一条17 kDa的条带。RBP仅在主带(17 kDa)检测到。在凹痕病2中,除主带外,ALB带和α1-m带呈高密度染色。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Electrophoretic analysis of various urinary proteins in Japanese patients with Dent disease
Patients with Dent disease excrete high amounts of urinary α1-microglobulin (α1-m), β2-microglobulin (β2-m), and retinol-binding protein (RBP). We found that the levels of α1-m, β2-m, and RBP in patients with Dent disease were higher than those in healthy subjects by 20 times, 130 times, and 200 times, respectively. These results confirmed the results of previous studies. The cellulose acetate membrane electrophoresis (CAE) showed that the characteristics of the urinary protein fraction in patients with Dent disease included appearance of pre-albumin, low albumin value, equal α1-globulin (α1-G) and α2-globulin (α2-G) values, disappearance of β-globulin (β-G), and appearance of slow α2-G (RBP) and slow β-G (β2-m). Urinary albumin (ALB), α1-m, β2-m, and RBP of patients with Dent disease 1 (mutation of CLCN 5) and Dent disease 2 (mutation of OCRL 1) and of healthy subjects were identified using western blot analysis after sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). Six bands from 84 kDa to 130 kDa were detected in addition to a main band of ALB (67 kDa). Seven bands from 51 kDa to 92 kDa were detected in addition to a main band of α1-m (34 kDa). A 17-kDa band was detected in addition to a main band of β2-m (14 kDa). RBP was detected only in the main band (17 kDa). The ALB and α1-m bands, which were in the high molecular weight range in addition to the main band, were densely stained in Dent disease 2.
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