Abraham D, Muir H, Olsen I, Winchester B
Biochem Biophys Res Commun. 1985 Jun 14;129(2):417-25. doi: 10.1016/0006-291x(85)90167-6.
Fibroblasts from patients with mannosidosis, the lysosomal storage disease resulting from an inherited deficiency of lysosomal alpha-D-mannosidase (EC 3.2.1.24), accumulate specific mannose-containing oligosaccharides which are characteristic of the disease (1,2). The present study shows that these substances were extensively degraded following transfer of the missing enzyme from normal lymphocytes to mannosidosis fibroblasts on direct contact in tissue culture. Moreover, prolonged correction of the metabolic abnormality of the recipient cells was sustained if contact with fresh donor lymphocytes was periodically renewed. These findings may be highly relevant to lymphocyte function in enzyme replacement therapy by transplantation procedures currently being attempted.
患有甘露糖苷贮积症（一种由溶酶体α-D-甘露糖苷酶遗传性缺乏引起的溶酶体贮积病）的患者的成纤维细胞会积累特定的含甘露糖寡糖，这些寡糖是该疾病的特征（1,2）。本研究表明，在组织培养中，当缺失的酶从正常淋巴细胞直接接触转移到甘露糖苷贮积症成纤维细胞时，这些物质会被大量降解。此外，如果定期更新与新鲜供体淋巴细胞的接触，受体细胞代谢异常的长期纠正就能持续。这些发现可能与目前正在尝试的移植程序进行酶替代治疗中的淋巴细胞功能高度相关。
