Cystinuria in dogs: comparison of the cystinuric component of the Fanconi syndrome in basenji dogs to isolated cystinuria.
Cystinuria in dogs: comparison of the cystinuric component of the Fanconi syndrome in basenji dogs to isolated cystinuria.
复制标题
狗的胱氨酸尿症：巴森吉犬范可尼综合征的胱氨酸尿成分与孤立性胱氨酸尿症的比较。
DOI：
10.1016/0026-0495(89)90173-x
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发表时间：
1989
期刊：
影响因子：
--
通讯作者：
Segal,S
中科院分区：
文献类型：
--
作者：
McNamara,PD;Rea,CT;Bovee,KC;Reynolds,RA;Segal,S
已经研究了两种胱氨酸尿症的动物模型：患有范可尼综合征的巴森吉犬和各种品种的胱氨酸结石形成犬。从这些动物身上分离出刷边膜，并对葡萄糖和胱氨酸的摄取进行了表征。对作为范可尼综合征组成部分的胱氨酸尿的巴森吉犬的分离刷边囊泡进行的实验显示，钠依赖性葡萄糖摄取减少，但胱氨酸摄取没有减少，尽管体内胱氨酸缺陷高达94%（即6%的重吸收）。相比之下，从胱氨酸结石形成犬（威尔士柯基犬）的肾脏中分离出的刷状边界囊泡，胱氨酸缺陷仅为16%（即84%的重吸收），与比格犬和巴森吉囊泡相比，胱氨酸的摄取减少。因此，在患有范可尼综合征的巴森吉犬中发现的胱氨酸尿症不同于典型的结石性胱氨酸尿症犬。导致患有范可尼综合征的巴森吉犬胱氨酸尿的改变似乎没有膜位点，可能反映了运输能量的改变，这在孤立的囊泡中未被检测到。胱氨酸缺陷在胱氨酸结石形成犬似乎反映在分离膜。
Two animal models for cystinuria have been examined: the Basenji dog with Fanconi syndrome and cystine stone-forming dogs of various breeds. Brush-border membranes were isolated from these animals and uptake ofd-glucose andl-cystine was characterized. Experiments with isolated brush-border vesicles from Basenji dogs with cystinuria as a component of the Fanconi syndrome showed diminished sodium-dependentd-glucose uptake but no decrease inl-cystine uptake even though the cystine defect in vivo was as high as 94% (ie, 6% reabsorption). In contrast, brush-border vesicles isolated from the kidney of a cystine stone-forming dog (Welsh Corgi) with a cystine defect of only 16% (ie, 84% reabsorption) had decreased uptake of cystine compared to values found for Beagle and Basenji vesicles. Thus, cystinuria found in Basenji dogs with the Fanconi syndrome differs from that in classic stone-forming cystinuric dogs. The alteration responsible for the cystinuria of Basenji dogs with Fanconi syndrome does not appear to have a membrane locus and may reflect altered energetics for transport, which are not detected in isolated vesicles. The cystine defect in cystinuric stone-forming dogs does appear to be reflected in the isolated membrane.