Abstract：
A remarkable enhancer of human glucocerebrosidase enzyme (GCase) was identified among a set of dihydroazulene-tagged iminosugars. An unprecedented 3.9-fold increase in GCase activity was detected on fibroblasts bearing the homozygous L444P mutation, which is frequently associated with neuronopathic Gaucher forms, and which commonly results refractory to chaperone-induced refolding.
摘要：
在一组二氢唑烯标记的亚氨基糖中鉴定出人类葡萄糖脑苷脂酶（GCase）的显着增强子。在带有纯合L444P突变的成纤维细胞上检测到GCase活性空前的3.9倍增加，这通常与神经病性戈谢型有关，通常导致伴侣诱导的重折叠难以抵抗。
