Charrow Joel
Children's Memorial Hospital, Division of Genetics, Birth Defects and Metabolism, Department of Pediatrics, Northwestern University, Feinberg School of Medicine, Chicago, Illinois 60614, USA.
Expert Opin Biol Ther. 2009 Jan;9(1):121-31. doi: 10.1517/14712590802573395.
Gaucher disease is the most common lysosomal storage disease, and the first lysosomal storage disease for which a specific therapy has been developed. Enzyme replacement therapy, with glucocerebrosidase purified from human placentae, was introduced in 1991. Recombinant human glucocerebrosidase, produced by Chinese hamster ovary cells in tissue culture, became available in 1994 and has replaced the placenta-derived product. These therapies have revolutionized the care of patients with type 1 Gaucher disease, reversing many of the pathological consequences of this disease, and preventing further progression. Furthermore, they have served as a model for the treatment of other lysosomal storage diseases and inborn errors of metabolism.
戈谢病是最常见的溶酶体贮积病,也是首个开发出特异性疗法的溶酶体贮积病。1991年引入了用从人胎盘中纯化的葡萄糖脑苷脂酶进行的酶替代疗法。1994年,通过组织培养的中国仓鼠卵巢细胞生产的重组人葡萄糖脑苷脂酶问世,并已取代了胎盘衍生产品。这些疗法彻底改变了1型戈谢病患者的治疗方式,扭转了该疾病的许多病理后果,并防止了病情的进一步发展。此外,它们还成为了治疗其他溶酶体贮积病和先天性代谢缺陷病的典范。