Kösel S, Matern D, Shin Y S
Children's Hospital, University of Munich, Federal Republic of Germany.
Eur J Pediatr. 1993;152 Suppl 1:S22-5. doi: 10.1007/BF02072083.
A multiple purification of phosphohydrolase (PH) and phosphotranslocase (PT) of the human liver microsomal glucose-6-phosphatase system has been obtained by a rapid two-step procedure using affinity chromatography. Sodium dodecyl sulphate polyacrylamide gel electrophoresis (SDS-PAGE) of the final products showed one major band each at 63 and 37 kDa for PH and PT respectively. The immunoblot analysis of SDS-PAGE of various purification steps for human liver using rabbit antibodies raised against the enzyme preparations also showed major bands at 63 and 37 kDa for PH and PT respectively. A major band at 260 kDa was observed by the Western blot of native PAGE of the enzyme preparation for PH. Cross-reacting materials at the positions of 63 and 37 kDa were detected only in liver, kidney and intestine. From five liver samples of patients suffering from type Ia glycogenosis there were diminished amounts of crossreacting materials at 63 kDa only in two samples. The uptake of glucose-6-phosphate has taken place in liposomes of Sepharose affinity purified products suggesting that this preparation may be a complex of PH and glucose-6-phosphate translocase.
通过使用亲和色谱的快速两步法,已对人肝微粒体葡萄糖-6-磷酸酶系统的磷酸水解酶(PH)和磷酸转运酶(PT)进行了多次纯化。最终产物的十二烷基硫酸钠聚丙烯酰胺凝胶电泳(SDS-PAGE)显示,PH和PT分别在63 kDa和37 kDa处各有一条主要条带。使用针对酶制剂产生的兔抗体对人肝的各种纯化步骤进行SDS-PAGE免疫印迹分析,结果也显示PH和PT分别在63 kDa和37 kDa处有主要条带。通过对PH酶制剂的天然PAGE进行蛋白质印迹法观察到在260 kDa处有一条主要条带。仅在肝脏、肾脏和肠道中检测到63 kDa和37 kDa位置的交叉反应物质。在患有Ia型糖原贮积病患者的五个肝脏样本中,只有两个样本中63 kDa处的交叉反应物质减少。葡萄糖-6-磷酸已在琼脂糖亲和纯化产物的脂质体中摄取,这表明该制剂可能是PH和葡萄糖-6-磷酸转运酶的复合物。