Roth K S, Spencer P D, Higgins E S, Spencer R F
Biochim Biophys Acta. 1985 Oct 24;820(1):140-6. doi: 10.1016/0005-2736(85)90225-1.
Succinylacetone, a catabolic end-product of tyrosine, is excreted in large quantities in urine from individuals with hereditary tyrosinemia and the Fanconi syndrome. Succinylacetone inhibits rat renal tubular concentrative uptake of the glucose transport analogue, methyl alpha-D-glucoside, in a noncompetitive and reversible fashion. This compound also depresses oxygen consumption by the rat renal tubule without fine structural damage to mitochondria. It is concluded that succinylacetone may be a useful probe in elucidation of the biochemical mechanism underlying the human Fanconi syndrome.
琥珀酰丙酮是酪氨酸的分解代谢终产物，在患有遗传性酪氨酸血症和范科尼综合征的个体尿液中大量排泄。琥珀酰丙酮以非竞争性和可逆的方式抑制大鼠肾小管对葡萄糖转运类似物α-D-甲基葡萄糖苷的浓缩摄取。该化合物还会降低大鼠肾小管的耗氧量，而不会对线粒体造成细微的结构损伤。结论是琥珀酰丙酮可能是阐明人类范科尼综合征潜在生化机制的有用探针。
