Kranhold J F, Loh D, Morris R C
Science. 1969 Jul 25;165(3891):402-3. doi: 10.1126/science.165.3891.402.
In patients with hereditary fructose intolerance, which is characterized by deficient aldolase activity toward fructose-1-phosphate, fructose induces a renal tubular dysfunction that implicates only the proximal convoluted tubule. Because normal metabolism of fructose by way of fructose-1-phosphate requires fructokinase, aldolase "B," and triokinase, the exclusively cortical location of these enzymes indicates that the medulla is not involved in the metabolic abnormality presumably causal of the renal dysfunction.
在遗传性果糖不耐受患者中，其特征是对1-磷酸果糖的醛缩酶活性不足，果糖会诱发仅累及近端曲管的肾小管功能障碍。由于果糖通过1-磷酸果糖的正常代谢需要果糖激酶、醛缩酶“B”和磷酸丙糖激酶，这些酶仅位于皮质表明髓质未参与可能导致肾功能障碍的代谢异常。
