Bovee K C, Segal S
Metabolism. 1984 Jul;33(7):602-7. doi: 10.1016/0026-0495(84)90056-8.
The renal reabsorption of cystine, lysine, arginine, and ornithine as well as other amino acids has been determined before and after lysine infusion in four normal and five cystinuric dogs. The large filtered load of lysine caused defective reabsorption of cystine in three of four normals and augmented the basal defect in all of the cystinuric dogs. Two of the cystinuric dogs responded with cystine clearances in excess of the glomerular filtration rate. The magnitude of increase of the reabsorptive defect for cystine observed after lysine infusion into the cystinuric dogs was unrelated to the extent of the basal defect. Two of the normal dogs and four of the five cystinuric dogs demonstrated defective arginine reabsorption after lysine loading, one of the cystinuric dogs having greater arginine excretion than the filtered load. Although normal dogs showed a moderate inability to reabsorb the large filtered load of lysine, three of the cystinuric dogs exhibited a -60 to -70% reabsorption, consistent with lysine secretion. Both normal and cystinuric dogs showed defective glycine absorption after lysine loading, but only cystinuric dogs showed variable defects in threonine, serine, histidine, methionine, and tyrosine when the basic amino acid was infused. Each cystinuric dog responded to lysine infusion in a different way, and the overall pattern of response differed from the normal, with evidence of induced secretion of cystine, lysine, and arginine in the affected dogs.
在4只正常犬和5只胱氨酸尿症犬中，测定了赖氨酸输注前后胱氨酸、赖氨酸、精氨酸、鸟氨酸以及其他氨基酸的肾脏重吸收情况。大量滤过的赖氨酸负荷导致4只正常犬中的3只胱氨酸重吸收缺陷，并加重了所有胱氨酸尿症犬的基础缺陷。2只胱氨酸尿症犬的胱氨酸清除率超过了肾小球滤过率。向胱氨酸尿症犬输注赖氨酸后观察到的胱氨酸重吸收缺陷增加的幅度与基础缺陷的程度无关。2只正常犬和5只胱氨酸尿症犬中的4只在赖氨酸负荷后表现出精氨酸重吸收缺陷，其中1只胱氨酸尿症犬的精氨酸排泄量超过滤过量。虽然正常犬对大量滤过的赖氨酸负荷有一定的重吸收能力，但3只胱氨酸尿症犬的重吸收率为-60%至-70%，与赖氨酸分泌一致。正常犬和胱氨酸尿症犬在赖氨酸负荷后均表现出甘氨酸吸收缺陷，但只有胱氨酸尿症犬在输注碱性氨基酸时，苏氨酸、丝氨酸、组氨酸、蛋氨酸和酪氨酸存在不同程度的缺陷。每只胱氨酸尿症犬对赖氨酸输注的反应都不同，总体反应模式与正常犬不同，受影响的犬有胱氨酸、赖氨酸和精氨酸诱导分泌的迹象。
