卟啉症中的酶异常。
Enzyme abnormalities in the porphyrias.
作者信息
Brodie M J, Moore M R, Goldberg A
出版信息
Lancet. 1977 Oct 1;2(8040):699-701. doi: 10.1016/s0140-6736(77)90507-4.
PMID:71507
Abstract
Evidence is presented that each of the porphyrias represents a different inborn error of metabolism in haem biosynthesis. Control of the pathway takes place by feedback repression and inhibition by haem of delta-aminolaevulinic-acid synthase. It is suggested that insituations where the activity of this enzyme is derepressed, prophobilinogen deaminase represents a secondary control step.
摘要
有证据表明，每种卟啉症都代表血红素生物合成中一种不同的先天性代谢缺陷。该途径的调控通过血红素对δ-氨基-γ-酮戊酸合酶的反馈抑制来实现。有人提出，在该酶活性去抑制的情况下，胆色素原脱氨酶代表一个次级调控步骤。
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