Smith S G, Jackson A H, Jackson T R
Ann Clin Res. 1976;8 Suppl 17:53-5.
Further evidence is presented for the enzymic conversion of protoporphyrinogen to protoporphyrin by observing the loss of 3H counts by 3H and 14C labelled coproporphyrinogen when incubated in chicken red cell haemolysates. Use was made of chemical and TLC fractionation of porphyrin extracts after incubation and a loss of half the 3H was again found. Evidence is presented for the role of harderoporphyrinogen as a natural intermediate in this conversion. The relevance of the findings in the porphyrias, and in particular variegate porphyria, is discussed. It is suggested that the enzymic process involved in the conversion of protoporphyrinogen to protoporphyrin may be deficient in variegate porphyria.
通过观察3H和14C标记的粪卟啉原在鸡红细胞溶血产物中孵育时3H计数的损失，进一步证明了原卟啉原通过酶促转化为原卟啉。孵育后对卟啉提取物进行化学和薄层层析分离，再次发现3H损失了一半。有证据表明硬卟啉原在这种转化中作为天然中间体的作用。讨论了这些发现与卟啉病，特别是杂合性卟啉病的相关性。有人提出，原卟啉原转化为原卟啉的酶促过程在杂合性卟啉病中可能存在缺陷。
