Gagnon R F, Somerville P, Thomson D M
Department of Medicine, Montreal General Hospital, McGill University, Montreal, P.Q., Canada.
Am J Nephrol. 1988;8(5):379-83. doi: 10.1159/000167621.
The circulating profile of beta-2-microglobulin (beta 2M) was determined in 8 end-stage renal disease patients on long-term dialysis (6 on hemodialysis, 2 on CAPD) by measuring beta 2M in different fraction after molecular sieve separation of their sera. Four patients had carpal tunnel syndrome with demonstrated amyloid in excised wrist tissues of which 2 were positive for beta 2M. In all patients despite very high blood levels (34.3-63.1 mg/l), beta 2M eluted exclusively as a single peak in the molecular weight region of about 12,000 daltons on a calibrated Sephacryl S-200 column. Recoveries from within the peak accounted for 96% of the applied beta 2M serum concentrations. These results were confirmed by molecular sieve separation of the enriched beta 2M-containing fractions by high-pressure liquid chromatography. We conclude that immunoreactive beta 2M in dialysis patients circulates as an intact monomer without evidence for the formation of aggregates or fragments. The pathogenesis of tissue deposition of this low-molecular-weight protein and its polymerisation to form a specific amyloid remains to be defined.
通过对8例长期透析的终末期肾病患者（6例血液透析，2例持续性不卧床腹膜透析）血清进行分子筛分离后，测定不同组分中的β2微球蛋白（β2M），以确定其循环情况。4例患者患有腕管综合征，切除的腕部组织中显示有淀粉样变，其中2例β2M呈阳性。在所有患者中，尽管血液中β2M水平非常高（34.3 - 63.1毫克/升），但在经校准的Sephacryl S - 200柱上，β2M仅作为一个单一峰洗脱，分子量约为12,000道尔顿。峰内回收率占所加β2M血清浓度的96%。通过高压液相色谱对富含β2M的组分进行分子筛分离，证实了这些结果。我们得出结论，透析患者体内具有免疫反应性的β2M以完整单体形式循环，没有形成聚集体或片段的证据。这种低分子量蛋白质在组织中的沉积及其聚合形成特定淀粉样变的发病机制仍有待确定。
