Hubert J J, O'Brien J S
Biochem J. 1983 Aug 1;213(2):473-8. doi: 10.1042/bj2130473.
The purification of dog liver acid beta-galactosidase is described. The dog enzyme migrated as a single major band on polyacrylamide-gel electrophoresis in the presence of sodium dodecyl sulphate, with a molecular weight of 60000. Antiserum raised against purified human liver acid beta-galactosidase cross-reacted with beta-galactosidase from dog liver, but not with those from cat liver or Escherichia coli. Tryptic peptide maps of the dog and human acid beta-galactosidases indicate that 21 of the 24 peptides observed were homologous; a similar result was obtained after chymotryptic peptide mapping. We conclude that dog and human acid beta-galactosidases are structurally similar, and that canine GM1 gangliosidosis (acid beta-galactosidase deficiency) is an excellent model for the same disease in man.
本文描述了犬肝酸性β-半乳糖苷酶的纯化过程。在十二烷基硫酸钠存在的情况下,该犬酶在聚丙烯酰胺凝胶电泳中迁移为单一主要条带,分子量为60000。用纯化的人肝酸性β-半乳糖苷酶制备的抗血清与犬肝β-半乳糖苷酶发生交叉反应,但与猫肝或大肠杆菌的β-半乳糖苷酶不发生交叉反应。犬和人酸性β-半乳糖苷酶的胰蛋白酶肽图谱表明,所观察到的24个肽中有21个是同源的;胰凝乳蛋白酶肽图谱分析后也得到了类似的结果。我们得出结论,犬和人酸性β-半乳糖苷酶在结构上相似,并且犬GM1神经节苷脂病(酸性β-半乳糖苷酶缺乏症)是人类同一种疾病的理想模型。