Heard G S, Tanner R W, Blevins T L, Evans J S, Redmond J B, Roth K S, Wolf B
Department of Human Genetics, Medical College of Virginia, Virginia Commonwealth University, Richmond 23298-0169.
Biochem Med Metab Biol. 1991 Feb;45(1):92-100. doi: 10.1016/0885-4505(91)90011-9.
Biotinidase activity was measured in plasmas of 1-, 7-, 14-, and 21-day-old rats from control dams and dams that had been fed a biotin-depleting diet from Day 15 of gestation. Biotinidase activity increased significantly in the plasma of rats from control and depleted mothers until Postnatal Day 14, after which there was a small but significant decline at Day 21. Differences between the mean activities of the two groups of pups on each sampling day were not significant and there were no significant differences in activity levels attributable to sex. Plasma albumin concentrations increased from birth until Day 21, and plasma biotinidase activity and albumin concentration were significantly correlated (r = +/- 0.43). We suggested that these two proteins may be controlled by a common mechanism in the early postnatal period, and that biotin deficiency does not affect the development of biotinidase activity. Because biotin-depleted neonatal pups show developmental changes in biotinidase activity similar to those of human newborns, and they can be produced reliably by depleting dams from Day 15 of gestation, they may be useful models for studying the developmental abnormalities associated with human biotinidase deficiency.
在来自对照母鼠以及从妊娠第15天开始喂食生物素耗竭饮食的母鼠所产的1日龄、7日龄、14日龄和21日龄大鼠的血浆中测量了生物素酶活性。来自对照母鼠和生物素耗竭母鼠的大鼠血浆中的生物素酶活性在出生后第14天之前显著增加,之后在第21天出现了小幅度但显著的下降。每组幼崽在每个采样日的平均活性之间的差异不显著,并且活性水平在性别上没有显著差异。血浆白蛋白浓度从出生到第21天增加,并且血浆生物素酶活性和白蛋白浓度显著相关(r = +/- 0.43)。我们认为这两种蛋白质可能在出生后早期受共同机制控制,并且生物素缺乏并不影响生物素酶活性的发育。由于生物素耗竭的新生幼崽表现出与人类新生儿相似的生物素酶活性发育变化,并且通过从妊娠第15天开始使母鼠生物素耗竭能够可靠地产生这些幼崽,它们可能是研究与人类生物素酶缺乏相关的发育异常的有用模型。