Tenhunen R
Department of Clinical Chemistry, University of Helsinki, Meilahti Hospital, Finland.
Scand J Clin Lab Invest Suppl. 1990;201:63-75.
The mechanism of regulation of heme biosynthesis is of great interest for both biological and medical reasons. The hepatic and erythropoietic porphyrias are shown to be hereditary or acquired disorders characterized by defects in specific enzymes of the heme biosynthetic pathway. In addition, several other diseases can be associated with a defect of heme biosynthesis. Heme has become well established in the treatment of acute porphyrias. In recent years, heme has been used increasingly for the treatment of other heme deficiency states as well.
出于生物学和医学两方面的原因，血红素生物合成的调节机制备受关注。肝性和红细胞生成性卟啉症被证明是遗传性或后天性疾病，其特征是血红素生物合成途径中特定酶的缺陷。此外，其他几种疾病也可能与血红素生物合成缺陷有关。血红素已在急性卟啉症的治疗中得到广泛应用。近年来，血红素也越来越多地用于治疗其他血红素缺乏状态。
