Harzer Klaus, Yildiz Yildiz
Biol Chem. 2015 Nov;396(11):1241-6. doi: 10.1515/hsz-2015-0144.
Beta-glucosidases (GBA) include GBA1, GBA2 and other β-glucosidases (non-GBA1-2). GBA1 is a lysosomal and GBA2 an extra-lysosomal enzyme. GBA1- and GBA2-deficient genetic conditions, with different phenotypes, are glucosylceramide (GC; the main GBA substrate) accumulating diseases. To study the activity profile of GBA, live fibroblasts were loaded with radioactive GC. The GC metabolism was measured in wild-type, GBA1-deficient (Gaucher disease) and GBA2-deficient (Gba2(-/- )mouse) cells. The differences found allowed the prediction of marked proportions of GBA1, GBA2, and particularly non-GBA1-2 (probably including GBA3, a cytosolic β-glucosidase) activity for wild-type cells. The high proportion of non-GBA1-2 suggests an important role of these enzymes.
β-葡萄糖苷酶（GBA）包括GBA1、GBA2和其他β-葡萄糖苷酶（非GBA1-2）。GBA1是一种溶酶体酶，GBA2是一种溶酶体外酶。GBA1和GBA2缺乏的遗传条件，具有不同的表型，是葡糖神经酰胺（GC；主要的GBA底物）蓄积性疾病。为了研究GBA的活性谱，用放射性GC加载活的成纤维细胞。在野生型、GBA1缺乏（戈谢病）和GBA2缺乏（Gba2(-/-)小鼠）细胞中测量GC代谢。所发现的差异使得能够预测野生型细胞中GBA1、GBA2，特别是非GBA1-2（可能包括GBA3，一种胞质β-葡萄糖苷酶）活性的显著比例。非GBA1-2的高比例表明这些酶具有重要作用。
