Results 141 to 150 of about 295,934 (166)
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13C-N.M.R. Studies on the pyrromethane cofactor of hydroxymethylbilane synthase

Tetrahedron Letters, 1988
By growing Escherichiacoli in the presence of 5-amino [5-13C] laevulinic acid, the enzyme hydroxymethylbilane synthase is produced carrying 13C-labels in its pyrromethane cofactor. It is then proved by 13C-n.m.r. spectroscopy that the cofactor is bound to the protein via the sulphur atom of a cysteine residue.
Uwe Beifuss   +3 more
openaire   +1 more source

19-Bromo-1-hydroxymethylbilane a novel inhibitor of Uro'gen III synthase

Bioorganic & Medicinal Chemistry, 1994
A novel hydroxymethylbilane analog, 19-Br-HMB (11), has been synthesized. Its activity with the enzyme Uro'gen III synthase shows competitive inhibition.
C, Pichon   +4 more
openaire   +2 more sources

Erythrocyte Hydroxymethylbilane Synthase Activity in a Chinese Family with Acute Intermittent Porphyria

Annals of Clinical Biochemistry: International Journal of Laboratory Medicine, 1988
Etude d'une famille chinoise avec analyse de l'activite enzymatique de l'uroporphyrinogen I synthase erythrocytaire et du porphobilinogen ...
F Y, Lee   +5 more
openaire   +2 more sources

A splicing mutation in the hydroxymethylbilane synthase gene in a Japanese family with acute intermittent porphyria

Clinical Biochemistry, 1999
Acute intermittent porphyria (AIP) is an autosomal dominant inherited disease caused by a decreased activity of hydroxymethylbilane synthase (HMBS). As far as the gene abnormalities of the HMBS, many different mutations have been reported. In this work, we investigated the presence of mutations in a Japanese family with AIP.A 44-year-old Japanese male ...
N, Maeda   +8 more
openaire   +2 more sources

Comparative inhibition of hepatic hydroxymethylbilane synthase by both hard and soft metal cations

Canadian Journal of Biochemistry and Cell Biology, 1984
The in vitro inhibition of hydroxymethylbilane synthase (EC 4.3.1.8, uroporphyrinogen I synthetase) obtained from livers of Sprague–Dawley rats has been studied with a wide range of di- and tri-valent metal ions. After purification by cell lysis, heat treatment, and centrifugation, the stable, soluble enzyme yielded sigmoidal inhibition curves with ...
D J, Farmer, B R, Hollebone
openaire   +2 more sources

Acute hepatic porphyrias: Identification of 46 hydroxymethylbilane synthase, 11 coproporphyrinogen oxidase, and 20 protoporphyrinogen oxidase novel mutations.

Molecular Genetics and Metabolism, 2019
The acute hepatic porphyrias (AHPs) are inborn errors of heme biosynthesis, which include three autosomal dominant porphyrias, Acute Intermittent Porphyria (AIP), Hereditary Coproporphyria (HCP), and Variegate Porphyria (VP), and the ultra-rare autosomal
Yonina Loskove   +5 more
semanticscholar   +1 more source

A new synthesis of porphobilinogen analogues, inhibitors of hydroxymethylbilane synthase.

Organic & biomolecular chemistry, 2004
Two analogues of porphobilinogen, the 6-methyl and 6,11-ethano derivatives, have been made by a new synthetic route and the 6-methyl analogue has proved to be the most potent inhibitor of hydroxymethylbilane synthase yet reported (Ki = 3 microM).
Raef, Ahmed, Finian J, Leeper
openaire   +1 more source

Purification of hydroxymethylbilane synthase from human erythrocytes

Biochemical Society Transactions, 1985
RICHARD C. BROWN   +2 more
openaire   +1 more source

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