Schröter J, Schott K J, Purtill M A, Neuhoff V
J Inherit Metab Dis. 1986;9(3):273-82. doi: 10.1007/BF01799660.
The influence of alpha-methylphenylalanine-induced hyperphenylalaninaemia (HYP) on the lysosomal protein degradation system in brain and liver of suckling rats was investigated. In both tissues cathepsin D and L activities, measured at 5, 10 and 15 days post partum (p.p.), exhibited no differences between experimental and control animals. N-Acetyl-beta-D-glucosaminidase (NAGase) activity in brain, measured at 10 and 15 days p.p., was not affected by HYP either. The release of valine and lysine from liver and brain homogenates respectively, serving as a measure for the lysosomal content of degradable proteins, was not influenced by HYP. Lysosomal integrity during incubation of homogenate was monitored by the recovery of NAGase activity in the cytosolic supernatant, and by the relative NAGase activity in total homogenates in the absence of the lysosome disrupting detergent Triton X-100. In conclusion, experimental HYP appears unlikely to influence the lysosomal protein degradation system in brain and liver of suckling rats.
研究了α-甲基苯丙氨酸诱导的高苯丙氨酸血症（HYP）对乳鼠脑和肝脏溶酶体蛋白降解系统的影响。在产后5、10和15天测量的实验动物和对照动物的组织中，组织蛋白酶D和L的活性均无差异。产后10天和15天测量的脑内N-乙酰-β-D-氨基葡萄糖苷酶（NAGase）活性也不受HYP影响。分别从肝脏和脑匀浆中释放缬氨酸和赖氨酸，作为可降解蛋白溶酶体含量的指标，也不受HYP影响。通过细胞溶质上清液中NAGase活性的恢复以及在不存在破坏溶酶体的去污剂Triton X-100的情况下总匀浆中相对NAGase活性，监测匀浆孵育期间的溶酶体完整性。总之，实验性HYP似乎不太可能影响乳鼠脑和肝脏的溶酶体蛋白降解系统。
