The "glucose effect" in erythropoietic protoporphyria.
The "glucose effect" in erythropoietic protoporphyria.
复制标题
红细胞生成性原卟啉症中的“葡萄糖效应”。
DOI：
10.1001/archinte.1968.03640050056011
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发表时间：
1968
影响因子：
--
通讯作者：
R. E. Sterling
中科院分区：
文献类型：
--
作者：
A. Redeker;R. E. Sterling
红细胞生成性原卟啉症（EPP）是一种遗传性光皮肤病，于1961年首次报道。1典型的综合征包括皮肤光敏性、红细胞和粪便原卟啉大量增加以及血浆原卟啉的可变但通常显著的增加。尿液正常。虽然光敏性显然是由于原卟啉，但这种卟啉的形成部位一直受到质疑。最初有人认为光敏性与高红细胞卟啉有关。然而，与其他皮肤卟啉病一致，随后证明了血浆中卟啉的增加。2，3 Redeker和Bryan在1964年提出了血浆卟啉引起光敏性并在非红细胞生成部位合成的可能性。4卟啉生物合成的限速酶是氨基乙酰丙酸合成酶（ALA-S）。肝ALA-S是急性间歇性卟啉病（AIP）患者的一种诱导酶，其合成速率与碳水化合物或蛋白质摄入量成反比
Erythropoietic protoporphyria (EPP) is an inherited photocutaneous disorder first described in 1961.1The typical syndrome includes cutaneous photosensitivity, greatly increased erythrocyte and fecal protoporphyrin, and a variable but often marked increase in plasma protoporphyrin. The urine is normal. Although the photosensitivity is clearly due to protoporphyrin, the site of formation of this porphyrin has been questioned. Initially it was suggested that the photosensitivity was associated with the high red cell porphyrin. However, in keeping with the other cutaneous porphyrias, increased porphyrin in the plasma was subsequently demonstrated.2,3The possibility that the plasma porphyrin causes the photosensitivity and is synthesized at a nonerythropoietic site was suggested by Redeker and Bryan in 1964.4 The rate-limiting enzyme for porphyrin biosynthesis is aminolevulinic acid synthetase (ALA-S). Hepatic ALA-S is an inducible enzyme in patients with acute intermittent porphyria (AIP) and its rate of synthesis is reciprocal with carbohydrate or protein intake