Maret A, Potier M, Salvayre R, Troly M, Beauregard G, Douste-Blazy L
Laboratoire de Biochimie Médicale, Faculté de Médecine, Toulouse, France.
Biochim Biophys Acta. 1989 Jul 6;996(3):254-6. doi: 10.1016/0167-4838(89)90255-0.
The genetic defect in Gaucher disease consists in a deficiency of a membrane-bound lysosomal acid beta-glucosidase. Using the radiation inactivation method, we have previously reported a subunit coupling of the mutated acid beta-glucosidase from Gaucher type 1 spleen in contrast to the normal one (Maret, A., Potier, M., Salvayre, R. and Douste-Blazy, L. (1983) FEBS Lett. 160, 93-97). We have used the same method to determine the effect of detergents on subunit coupling or uncoupling of acid beta-glucosidase in normal and Gaucher spleens. The hypothesis that detergent activation of beta-glucosidase could be due to subunit association or dissociation has been tested. The radiation inactivation size of beta-glucosidase in absence of detergent was 71,000 and 135,500 for normal and Gaucher spleen, respectively, whereas the corresponding values in presence of detergent were 84,000 and 169,000. The higher values obtained in the presence of detergent are incompatible with association or dissociation of subunits but correspond to the increase generally observed for proteins irradiated in the presence of Triton X-100.
戈谢病的基因缺陷在于膜结合溶酶体酸性β-葡萄糖苷酶缺乏。我们之前采用辐射失活法报道了，与正常的酸性β-葡萄糖苷酶相比，来自1型戈谢病脾脏的突变酸性β-葡萄糖苷酶存在亚基偶联（马雷，A.，波捷，M.，萨尔韦尔，R.和杜斯特-布拉齐，L.（1983年）《欧洲生物化学学会联合会快报》160，93 - 97）。我们使用相同方法来确定去污剂对正常脾脏和戈谢病脾脏中酸性β-葡萄糖苷酶亚基偶联或解偶联的影响。已对去污剂激活β-葡萄糖苷酶可能是由于亚基缔合或解离这一假说进行了检验。在不存在去污剂的情况下，正常脾脏和戈谢病脾脏中β-葡萄糖苷酶的辐射失活大小分别为71,000和135,500，而在存在去污剂的情况下，相应值分别为84,000和169,000。在存在去污剂时获得的较高值与亚基的缔合或解离不相符，但与在Triton X - 100存在下照射的蛋白质通常观察到的增加相对应。
