hydroxymethylbilane

tetrapyrrole intermediate in pigment biosynthesis by Euglena gracilis; universal intermediate in biosynthesis of uroporphyrinogen III; structure given in first source

Also Known As:

Networked: 8 relevant articles (0 outcomes, 0 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Aguilera, Paula: 1 article (03/2022)
2.	Casals, Gregori: 1 article (03/2022)
3.	Gomez-Gomez, Alex: 1 article (03/2022)
4.	Langohr, Klaus: 1 article (03/2022)
5.	Marcos, Josep: 1 article (03/2022)
6.	Pavon, Cristina: 1 article (03/2022)
7.	Pozo, Oscar J: 1 article (03/2022)
8.	To-Figueras, Jordi: 1 article (03/2022)
9.	Aarsand, Aasne K: 1 article (07/2019)
10.	Andersen, Janice: 1 article (07/2019)

Related Diseases

1.	Acute Intermittent Porphyria (Porphyria, Acute) 03/16/2022 - "Acute intermittent porphyria (AIP) is an inherited rare hepatic disorder due to mutations within the hydroxymethylbilane gene. " 06/05/2015 - "Acute intermittent porphyria (AIP) is an autosomal-dominant hepatic disorder caused by the half-normal activity of hydroxymethylbilane (HMB) synthase. " 07/03/2019 - "Acute intermittent porphyria (AIP) is an inherited metabolic disease with low clinical penetrance caused by mutations in the hydroxymethylbilane (HMBS) gene. " 03/01/2023 - "Acute intermittent porphyria (AIP) is an autosomal dominant disorder of heme biosynthesis in the liver that is caused by the accumulation of toxic heme metabolites aminolevulinic acid (ALA) and porphobilinogen (PBG) due to a deficiency in the enzyme hydroxymethylbilane synthase (HMBS). "
2.	Erythropoietic Porphyria (Congenital Erythropoietic Porphyria) 04/10/1995 - "Uroporphyrinogen-III synthase (URO-S; EC 4.2.1.75), the fourth enzyme in the heme biosynthetic pathway, is responsible for the conversion of hydroxymethylbilane to the cyclic tetrapyrrole, uroporphyrinogen III. The deficient activity of URO-S is the enzymatic defect in congenital erythropoietic porphyria (CEP), an autosomal recessive disorder. " 10/01/1988 - "Uroporphyrinogen III synthase [URO-synthase; hydroxymethylbilane hydro-lyase (cyclizing), EC 4.2.1.75], the fourth enzyme in the heme biosynthetic pathway, is responsible for conversion of the linear tetrapyrrole, hydroxymethylbilane, to the cyclic tetrapyrrole, uroporphyrinogen III. The deficient activity of URO-synthase is the enzymatic defect in the autosomal recessive disorder congenital erythropoietic porphyria. "
3.	Skin Diseases (Skin Disease) 03/13/2008 - "The results allow us to discard the sigmatropic shift mechanism and suggest that the D-ring of the hydroxymethylbilane substrate binds to the enzyme in a conformation that shields its terminal portion from reacting with ring A and prevents the formation of the biologically useless uroporphyrinogen I, whose accumulation (in individuals lacking functional uroporphyrinogen III synthase) leads to severe cutaneous dermatosis."
4.	Neoplasms (Cancer) 11/01/2006 - "Eleven reference genes (18s ribosomal ribonucleic acid [RNA], 28s ribosomal RNA, ubiquitin, beta-actin, glycerine aldehyde dehydrogenase, ATP-synthase subunit 5B, hydroxymethyl-bilane synthase, hypoxanthine-phosphoribosyl transferase, ribosomal protein L32, tryptophan 5-monooxygenase activation protein (zeta polypeptide), and TATA-Box binding protein) were analyzed in use as references for gene expression profiling experiments using quantitative reverse transcription polymerase chain reaction (qRT-PCR) in canine mammary tumors. "
5.	Congenital, Hereditary, and Neonatal Diseases and Abnormalities (Congenital Disorders) 10/01/1988 - "Uroporphyrinogen III synthase [URO-synthase; hydroxymethylbilane hydro-lyase (cyclizing), EC 4.2.1.75], the fourth enzyme in the heme biosynthetic pathway, is responsible for conversion of the linear tetrapyrrole, hydroxymethylbilane, to the cyclic tetrapyrrole, uroporphyrinogen III. The deficient activity of URO-synthase is the enzymatic defect in the autosomal recessive disorder congenital erythropoietic porphyria. "

Related Drugs and Biologics

1.	Enzymes
2.	Uroporphyrinogen III Synthetase (Uroporphyrinogen-III Synthase)
3.	Heme (Haem)
4.	Tetrapyrroles
5.	Uroporphyrinogens
6.	TATA-Box Binding Protein (TATA-Binding Protein)
7.	Ubiquitin
8.	Hypoxanthine
9.	Tryptophan (L-Tryptophan)
10.	Transferases