C1型尼曼-匹克病小鼠生长繁殖及血液生理生化指标的测定分析

Analysis of growth,reproductive and blood physiological and biochemical parameters in Niemann-Pick disease type C1 mice

目的测定SPF级C1型尼曼-匹克病小鼠(Npc1^(-/-)小鼠)的生长繁殖及血液生理生化指标,为开展NPC1病人的研究提供理论性的基础资料。方法 (1)选取0~77日龄Npc1^(-/-)、Npc1^(+/-)和Npc1+/+小鼠雌雄各40只,定期称重并绘制生长曲线;(2)Npc1^(-/-)小鼠由Npc1^(+/-)小鼠交配繁殖产生,统计Npc1^(+/-)小鼠连续4代的繁殖数据;(3)检测60日龄Npc1^(-/-)和Npc1+/+小鼠的血液生理生化指标。结果 (1)Npc1^(-/-)小鼠的体重在7周前随着日龄的增加而增加,7周后随着日龄的增加而减少,直至11周左右死亡。Npc1+/+和Npc1^(+/-)小鼠的体重均随着日龄的增长而逐渐增加,两者之间并没有显著差别。4周后雄性比雌性体重增加明显比雌性小鼠快;(2)Npc1^(+/-)小鼠不同代内交配分娩间隔、窝产仔数、离乳仔数、离乳仔数中雌雄数量及阳性数量差异无显著性(P>0.05),而第2代的离乳率明显大于第1代(P<0.05);(3)Npc1^(-/-)和Npc1+/+小鼠血液生理指标中平均红细胞血红蛋白含量(MCH)和平均过氧化物酶指数(MPXI)差异有显著性(P<0.05),其余差异无显著性(P>0.05)。生化指标中尿素(UREA)差异有极显著性(P<0.01),而天门冬氨酸氨基转移酶(AST)、葡萄糖(GLU)、乳酸脱氢酶(LDH)、钾(K)和铜(Cu)等差异有显著性(P<0.05),其余差异无显著性(P>0.05)。结论 (1)Npc1^(-/-)、Npc1^(+/-)和Npc1+/+小鼠的生长曲线因基因型和性别的不同而存在差异;(2)Npc1^(+/-)小鼠不同代的繁殖能力差异无显著性;(3)Npc1^(-/-)和Npc1+/+小鼠的部分血液生理生化指标差异有显著性。

Objective To investigate the growth curve,breeding rate,and blood physiological and biochemical parameters in Npc1 gene mutant mice（ Npc1-/-） for providing theoretical evidence in research on Niemann-Pick disease type C1（ NPC1） patient. Methods 1） The body mass of Npc1-/-,Npc1＋/-,and Npc1＋/＋mice（ n = 120; 60♀,60♂） was measured from 0 to 77 days;（ 2） As Npc1-/-mice were born only by the mating Npc1＋/-mice,the breeding rate of Npc1＋/-mice was counted here from the 1st to 4th generation;（ 3） The blood physiological and biochemical parameters were measured on both Npc1-/-and Npc1＋/＋mice at 60 days. Results 1） Compared with the wild type controls,thebody weight of Npc1-/-mice was progressively increased up to 7 weeks and then decreased,and died around 11 weeks.The body weight of the Npc1＋/-and Npc1＋/＋mice was increased as time went on. After 4 weeks,the male mice showed a higher weight gain than the females;（ 2） The generations of Npc1＋/-mice had no significant difference in mating-parturition interval,litter size,weaning litter and the number of male and female（ P 〉 0. 05）,but the weaning rate of the 2nd generation was significantly higher than that of the 1st generation（ P 〈 0. 05）;（ 3） The hematological parameters showed a significant difference only in mean corpuscular hemoglobin（ MCH） and mean peroxidase index（ MPXI） between the Npc1-/-and Npc1＋/＋mice（ P 〈 0. 05）. No significant difference was found in other hematological parameters（ P 〉0. 05）. Among the biochemical parameters,aspartate aminotransferase（ AST）,glucose（ GLU）,lactate dehydrogenase（ LDH）,potassium（ K） and copper（ Cu） had a significant difference between the Npc1-/-and Npc1＋/＋mice（ P 〈0. 05）. Conclusions 1） The growth curves of Npc1-/-,Npc1＋/-,and Npc1＋/＋mice are different due to different genotype and sex;（ 2） The reproduction rates of Npc1＋/-mice have no significant difference among different generations;（ 3）The blood physiological parameters（ MCH,MPXI） and biochemical parameters（ UREA,AST,GLU,LDH,K,Cu） are significantly different between Npc1-/-and Npc1＋/＋mice.