原发性果糖尿症:红细胞中果糖3-磷酸水平升高。

Enzyme Pub Date : 1992-01-01 DOI:10.1159/000468811

A Petersen, B Steinmann, R Gitzelmann

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引用次数: 5

摘要

3名患有原发性果糖尿症的成年兄弟姐妹的红细胞中含有45-200 mumol/l的果糖3-磷酸(Fru-3-P)，即正常对照浓度的3-15倍。山梨醇3-磷酸含量也有所增加，但增加的程度较轻。口服50克果糖，5小时后红细胞Fru-3-P增加40 μ mol/l。体外红细胞Fru-3-P形成率正常。HbA1、HbA1c正常。Fru-3-P在糖尿病并发症中的致病作用受到质疑。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Essential fructosuria: increased levels of fructose 3-phosphate in erythrocytes.

Erythrocytes of 3 adult siblings with essential fructosuria contained 45-200 mumol/l fructose 3-phosphate (Fru-3-P), i.e. 3-15 times the concentration in normal controls. Sorbitol 3-phosphate was also increased, but to a lesser degree. An oral load with 50 g of fructose produced an additional 40 mumol/l increase of erythrocyte Fru-3-P after 5 h. The rate of Fru-3-P formation by red cells in vitro was normal. HbA1 and HbA1c were normal. The suspected pathogenetic role of Fru-3-P in diabetic complications is questioned.

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Enzyme

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