Sato H, Keino H, Aono S, Semba R, Kashiwamata S
J Neurochem. 1987 Jun;48(6):1823-5. doi: 10.1111/j.1471-4159.1987.tb05742.x.
Activities of six lysosomal enzymes in the cerebellum of jaundiced homozygous (jj) Gunn rats were examined from 5 to 20 days of life and compared with those in heterozygotes (j+). Significantly higher enzyme activities were first detected at 8 days. The jj/j+ activity ratios of all enzymes peaked at 15 days. The ratios of beta-glycerophosphatase, beta-mannosidase, and acid lipase were only 1.3-1.7, whereas those of arylsulfatase and cathepsin were 2.0 and 3.1, respectively. The most striking increase in activity was observed with beta-glucuronidase, the ratio of which was 8.4. These results indicate a selective increase in activities of certain lysosomal enzymes in the hypoplastic cerebellum of jj rats.
在出生5至20天的黄疸纯合子（jj）冈恩大鼠的小脑中检测了六种溶酶体酶的活性，并与杂合子（j+）的酶活性进行了比较。在第8天首次检测到酶活性显著升高。所有酶的jj/j+活性比值在第15天达到峰值。β-甘油磷酸酶、β-甘露糖苷酶和酸性脂肪酶的比值仅为1.3-1.7，而芳基硫酸酯酶和组织蛋白酶的比值分别为2.0和3.1。β-葡萄糖醛酸酶的活性增加最为显著，其比值为8.4。这些结果表明，jj大鼠发育不全的小脑中某些溶酶体酶的活性有选择性增加。
