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本文常用外文缩写:AIP 急性间歇性卟啉病ALA σ-氨基左旋酸(卟啉原前躯物质)ALA-S σ-氨基-γ-戊酮酸合成酶,限速酶HCP 混合型卟啉病Heme 正铁血红素PCT 迟发性皮肤型卟啉病,症状性卟啉病PBG 卟胆原VP 变异型卟啉病卟啉在人体代谢中起着重要影响。过去有偏重于卟啉代谢与血红素的关联,近来则认为卟啉同肝脏代谢有更紧密的关系。肝脏是合成及利用卟啉的主要部位,亦是卟啉代谢异常时病变的好发部位。肝病的存在可促使某些原发性卟啉病表现加剧,在症状性或继发性卟啉病中,肝病是其最常见病因之一。
This article commonly used foreign abbreviations: AIP acute intermittent porphyria ALA Sigma-aminolevulinic acid (porphyrin precursor substance) ALA-S σ-amino-γ-pentoketolates synthase, rate-limiting enzyme HCP mixed porphyria Heme Heme Hematopoietin PCT Delayed cutaneous porphyria, Symptomatic porphyria disease PBG Porphyromoblastin VP Mutant porphyria Porphyrin plays an important role in human metabolism. In the past, emphasis was placed on the association of porphyrin metabolism with heme, which has recently been suggested to have a closer relationship with liver metabolism. The liver is the main part of the synthesis and utilization of porphyrin, is also a predilection site for dysplasia of porphyria. The presence of liver disease can promote the performance of some of the primary porphyria disease, liver disease is one of the most common cause of symptomatic or secondary porphyria.