Results 181 to 190 of about 256,046 (232)

Computational modeling of the catalytic mechanism of hydroxymethylbilane synthase.

Physical Chemistry Chemical Physics, 2019
Hydroxymethylbilane synthase (HMBS), the third enzyme in the heme biosynthesis pathway, catalyzes the formation of 1-hydroxymethylbilane (HMB) by a stepwise polymerization of four molecules of porphobilinogen (PBG) using the dipyrromethane (DPM) cofactor.
Navneet Bung, Navneet Bung, Arijit Roy, U. Priyakumar, Gopalakrishnan Bulusu, Gopalakrishnan Bulusu   +5 more
semanticscholar   +3 more sources

Network analysis of hydroxymethylbilane synthase dynamics.

Journal of Molecular Graphics and Modelling, 2020
Hydroxymethylbilane synthase (HMBS) is one of the key enzymes of the heme biosynthetic pathway that catalyzes porphobilinogen to form the linear tetrapyrrole 1-hydroxymethylbilane through four intermediate steps. Mutations in the human HMBS (hHMBS) can lead to acute intermittent porphyria (AIP), a lethal metabolic disorder.
Broto Chakrabarty, Dibyajyoti Das, Navneet Bung, Arijit Roy, Gopalakrishnan Bulusu   +4 more
semanticscholar   +3 more sources

Hydroxymethylbilane synthase (aka porphobilinogen deaminase): a novel metabolic tumor suppressor gene in hepatocellular carcinoma.

Journal of Hepatology, 2022
A. Fontanellas, M. Avila
semanticscholar   +3 more sources

Identification and molecular analysis of 17 novel variants of hydroxymethylbilane synthase in Chinese patients with acute intermittent porphyria

Clinical Genetics, 2021
A partial deficiency of the heme biosynthetic enzyme hydroxymethylbilane synthase (HMBS) leads to acute intermittent porphyria (AIP), a severe neurovisceral, autosomal dominant disorder with low penetrance. Even though in‐depth investigations of the HMBS
Yuanxiang Hu, Wei-hong Li, Ninglin Kang, M. Liyan, Teng Qing, Guiling Mo, Jiahong Wu, Xinyang Wang, Renjie Bi, Song-yun Zhang   +9 more
semanticscholar   +1 more source

Functional studies of rat hydroxymethylbilane synthase

Bioorganic Chemistry, 2008
The structurally related tetrapyrrolic pigments are a group of natural products that participate in many of the fundamental biosynthetic and catabolic processes of living organisms. Hydroxymethylbilane synthase catalyzes a rate-limiting step for the biosyntheses of tetrapyrrolic natural products.
Nan, Li, Xiusheng, Chu, Long, Wu, Xiaojun, Liu, Ding, Li   +4 more
openaire   +2 more sources

Hydroxymethylbilane synthase: complete genomic sequence and amplifiable polymorphisms in the human gene.

Genomics, 1993
Acute intermittent porphyria (AIP), an autosomal dominant inborn error of heme biosynthesis, results from the half-normal activity of the heme biosynthetic enzyme hydroxymethylbilane synthase (HMB-synthase). Heterozygous individuals are prone to life-threatening acute neurologic attacks, which are precipitated by certain drugs and other metabolic ...
H. Yoo, C. A. Warner, Chia-hsiang Chen, R. Desnick   +3 more
semanticscholar   +3 more sources

Interaction of 5-hydroxymethyl-furfural with hydroxymethylbilane synthase

Phytochemistry, 1997
Abstract The inhibition of chlorophyll biosynthesis in greening cress seedlings (Lepidium sativum L.) by 5-hydroxymethylfurfural (5-HMF), a natural compound isolated from the bulbs of Gladiolus spp. was investigated in vitro and in vivo. A direct reaction between 5-HMF and the chlorophyll precursor porphobilinogen was observed at pH 1.0 but not at pH
Joachim Wübert, Ulrike Oster, Wolfhart Rüdiger   +2 more
openaire   +1 more source

Time-resolved and static-ensemble structural chemistry of hydroxymethylbilane synthase

Faraday Discuss., 2003
The enzyme hydroxymethylbilane synthase (HMBS, EC 4.3.1.8), 313 amino acid residues and MW 34 kDa, also known as porphobilinogen deaminase (PBGD), catalyses the stepwise polymerization of four molecules of porphobilinogen (PBG) to the linear tetrapyrrole 1-hydroxymethylbilane. Several crystallographic structures of HMBS have been previously determined,
John R, Helliwell, Yeu Perng, Nieh, Jarjis, Habash, Paul F, Faulder, James, Raftery, Michele, Cianci, Michael, Wulff, Alfons, Hädener   +7 more
openaire   +2 more sources

Hydroxymethylbilane Synthase Gene Mutations and Polymorphisms in Brazilian Families with Acute Intermittent Porphyria

Annals of Human Genetics, 2015
SummaryAcute intermittent porphyria (AIP), an autosomal dominant disorder, is caused by a deficiency of hydroxymethylbilane synthase (HMBS). In the present study, we sought to establish a correlation between HMBS activity with the presence of mutations and polymorphisms.
A. Gonzaga, Lidia M F Amorim, A. M. Fonseca, T.L.S. Nogueira, Olga Maria Diniz Pereira, M. Nagai, Orlando Cesar Oliveira Barretto, G. S. Ribeiro   +7 more
semanticscholar   +3 more sources

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, B P, Atshaves, R, Danso-Danquah, N J, Stolowich, A I, Scott   +4 more
openaire   +2 more sources