Sidransky H, Verney E
J Exp Pathol. 1985 Spring;2(1):47-57.
Young rats were force-fed a lysine + arginine-devoid diet or a complete diet for 3 days, and selected biochemical and morphologic studies were conducted. Rats force-fed the experimental diet in comparison with those force-fed the control diet for 3 days showed decreased body weight gain, hepatomegaly with periportal fatty liver, pancreatic and splenic atrophy, and enhanced 14C-leucine incorporation into hepatic proteins. Differences in the experimental animals were observed in the free amino acid levels of serum (decreased lysine, arginine, and ornithine) and liver (decreased ornithine), in blood chemistries (decreased levels of ammonia N2, uric acid, cholesterol, protein, albumin, alkaline phosphatase, LDH and SGOT) and in hematologic findings (leukocytopenia and thrombocytopenia after a morning feeding). The experimental findings in young rats force-fed the lysine + arginine-devoid diet were compared with those reported to develop in children with lysinuric protein intolerance (LPI), an autosomal recessive defect in diamino acid transport. Children with LPI as described by others reveal a number of similarities as well as a number of differences in comparison to the findings in the experimental animals. The comparison suggests that some of the pathological manifestations of LPI may be related to a deficiency of diamino acids but others must be due to different alterations in this complex human disease.
将幼鼠强制喂食缺乏赖氨酸 + 精氨酸的饲料或完全饲料3天，并进行了选定的生化和形态学研究。与强制喂食对照饲料3天的大鼠相比，强制喂食实验饲料的大鼠体重增加减少、肝脏肿大伴门静脉周围脂肪肝、胰腺和脾脏萎缩，以及14C - 亮氨酸掺入肝蛋白增加。在实验动物中观察到血清（赖氨酸、精氨酸和鸟氨酸水平降低）和肝脏（鸟氨酸水平降低）的游离氨基酸水平、血液化学指标（氨氮、尿酸、胆固醇、蛋白质、白蛋白、碱性磷酸酶、乳酸脱氢酶和谷草转氨酶水平降低）以及血液学检查结果（晨饲后白细胞减少和血小板减少）存在差异。将强制喂食缺乏赖氨酸 + 精氨酸饲料的幼鼠的实验结果与据报道患有赖氨酸尿性蛋白不耐受症（LPI，一种二氨基酸转运的常染色体隐性缺陷）的儿童的实验结果进行了比较。其他人描述的患有LPI的儿童与实验动物的结果相比，既有一些相似之处，也有一些不同之处。这种比较表明，LPI的一些病理表现可能与二氨基酸缺乏有关，但其他表现肯定是由于这种复杂的人类疾病中的不同改变所致。
