Bovée K C, Bush M, Dietz J, Jezyk P, Segal S
Science. 1981 May 22;212(4497):919-20. doi: 10.1126/science.7233184.
Of 42 maned wolves in zoos or live-trapped in Brazil, 34 had excessive cystine in their urine. Renal clearance studies of five of the affected wolves revealed a variable defect for the reabsorption of cystine and dibasic amino acids. The renal tubular handling of other solutes including glucose, phosphate, sodium, potassium, and uric acid was considered normal. Urinary calculi composed of cystine were found in four wolves and proved fatal in three of them. With the exception of the high incidence in this species, this hereditary disease resembles the disorder described in dogs and humans.
在巴西动物园圈养或活体捕获的42只鬃狼中,有34只尿液中胱氨酸过量。对其中5只患病鬃狼的肾脏清除率研究显示,它们对胱氨酸和二碱基氨基酸的重吸收存在不同程度的缺陷。肾小管对包括葡萄糖、磷酸盐、钠、钾和尿酸在内的其他溶质的处理被认为是正常的。在4只鬃狼中发现了由胱氨酸组成的尿结石,其中3只因此死亡。除了该物种的高发病率外,这种遗传性疾病与在狗和人类中描述的疾病相似。