Sanfilippo D syndrome: Correction of glucosamine-6-sulphatase deficiency following fibroblast culture in chang's media

The de-O-sulphation of α-linked glucosamine-6-sulphate residues in heparan sulphate requires a specific sulphatase, glucosamine-6-sulphatase, which has been shown to be deficient in tissues of Sanfilippo D, or mucopolysaccharidosis type IIID (MPS IIID), patients. MPS IIID fibroblasts cultured in Basal Eagle's medium supplemented with either fetal calf serum or heat-inactivated fetal calf serum, MDCB or Ultraserg media had residual glucosarnine-6-sulphatase activities towards a heparin-derived trisaccharide substrate, O-(α-N-acetylglucosamine-6-sulphate)-(1→4)-L -O-(α-iduronic acid-2-sulphate)-(1→4)-D -O-2,5-anhydro [1-³H]mannitol-6-sulphate, GlcNAc6S-IdoA2S-anM6S, which were less than 1 per cent of the normal range for fibroblasts cultured in Basal Eagle's medium supplemented with fetal calf serum. However, the glucosamine-6-sulphatase activities of MPS IIID fibroblasts grown in Chang's medium were similar to the activities in normal control fibroblasts which were cultured in Basal Eagle's medium. These results indicate that caution is required for prenatal diagnosis of MPS IIID patients using chorionic villi or amniotic cells cultured in Chang's medium.     

高氨垃圾渗滤液的吹脱条件控制研究

采用吹脱塔处理含有高浓度氨氮的垃圾渗滤液,研究了操作参数的控制对吹脱效率的影响.结果表明,当pH≤11.0时,气水比决定了高径比对吹脱效率的影响,气水比较低时,高径比越小,吹脱效率越高;气水比较高时,高径比越大,吹脱效率越高;当pH过高时,高径比对吹脱效率没有影响.初始pH和温度相互影响,pH越低时,温度对吹脱效率的影响越显著;温度越低时,初始pH对吹脱效率的影响越显著.当pH≥10.4时,出水pH和残余氨氮浓度呈指数关系,可以作为反应吹脱运行状况的指示参数或控制参数.合理控制pH和气水比并实时监控出水pH是获得稳定、经济的吹脱效果的关键.