Spencer P D, Roth K S
Biochem Med Metab Biol. 1987 Feb;37(1):101-9. doi: 10.1016/0885-4505(87)90015-6.
Infants with hereditary tyrosinemia also have a renal Fanconi syndrome and excrete succinylacetone (SA). We have studied the effects of SA on rat renal tubular amino acid transport in vivo and in vitro using isolated renal tubules. Injection of SA produces increased clearance of several amino acids in the intact animal. In vitro SA causes a reversible inhibition of alpha-aminoisobutyric acid uptake, resulting from depressed low- and high-affinity transport systems. Addition of glutamate, succinate, or glucose, alone or in combination, did not restore transport. These observations suggest the usefulness of SA in the production of a physiologic animal model for the study of the human Fanconi syndrome.
患有遗传性酪氨酸血症的婴儿也会出现肾性范科尼综合征，并排泄琥珀酰丙酮（SA）。我们利用分离的肾小管，在体内和体外研究了SA对大鼠肾小管氨基酸转运的影响。给完整动物注射SA会使几种氨基酸的清除率增加。在体外，SA会导致α-氨基异丁酸摄取的可逆性抑制，这是由低亲和力和高亲和力转运系统的抑制所致。单独或联合添加谷氨酸、琥珀酸或葡萄糖并不能恢复转运。这些观察结果表明，SA在建立用于研究人类范科尼综合征的生理性动物模型方面具有实用性。
