doi: 10.1016/s0140-6736(77)90507-4.
Enzyme abnormalities in the porphyrias
- PMID: 71507
- DOI: 10.1016/s0140-6736(77)90507-4
Item in Clipboard
Enzyme abnormalities in the porphyrias
Lancet.
.
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.
Similar articles
-
The treatment of acute intermittent porphyria with laevulose.Clin Sci Mol Med. 1977 Oct;53(4):365-71. doi: 10.1042/cs0530365. Clin Sci Mol Med. 1977. PMID: 913061
-
Sideroblastic anemia.Adv Intern Med. 1977;22:229-49. Adv Intern Med. 1977. PMID: 320843 Review. No abstract available.
-
Relationships between acute hepatic porphyrias due to genetic variability of primary enzyme defects and limiting function of uroporphyrinogen synthase.Int J Biochem. 1978;9(12):911-6. doi: 10.1016/0020-711x(78)90069-1. Int J Biochem. 1978. PMID: 744295 No abstract available.
-
The biochemistry of the porphyrins.Clin Haematol. 1980 Jun;9(2):227-52. Clin Haematol. 1980. PMID: 6994963 Review. No abstract available.
-
Alcohol and porphyrin metabolism.Alcohol Alcohol. 2000 Mar-Apr;35(2):109-25. doi: 10.1093/alcalc/35.2.109. Alcohol Alcohol. 2000. PMID: 10787385 Review.
Cited by
-
Ferrochelatase and N-alkylated porphyrins.Mol Cell Biochem. 1984 Sep;64(2):127-37. doi: 10.1007/BF00224769. Mol Cell Biochem. 1984. PMID: 6390167 Review.
-
The effects of chronic carbamazepine treatment of haem biosynthesis in man and rat.Eur J Clin Pharmacol. 1988;35(3):241-7. doi: 10.1007/BF00558260. Eur J Clin Pharmacol. 1988. PMID: 3181278
-
The inherited enzymatic defect in porphyria variegata.Hum Genet. 1981;58(4):425-8. doi: 10.1007/BF00282829. Hum Genet. 1981. PMID: 7327566
-
Antipyrine metabolism in acute hepatic porphyria in relapse and remission.Br J Clin Pharmacol. 1987 Mar;23(3):358-61. doi: 10.1111/j.1365-2125.1987.tb03059.x. Br J Clin Pharmacol. 1987. PMID: 3567053 Free PMC article.
-
Effect of stanozolol on delta-aminolaevulinic acid synthase and hepatic monooxygenase activity in man and rat.Eur J Clin Pharmacol. 1984;26(5):587-90. doi: 10.1007/BF00543490. Eur J Clin Pharmacol. 1984. PMID: 6468473
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
