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Uroporphyrinogen III Synthetase (Uroporphyrinogen-III Synthase)

An enzyme that catalyzes the cyclization of hydroxymethylbilane to yield UROPORPHYRINOGEN III and water. It is the fourth enzyme in the 8-enzyme biosynthetic pathway of HEME, and is encoded by UROS gene. Mutations of UROS gene result in CONGENITAL ERYTHROPOIETIC PORPHYRIA.
Also Known As:
Uroporphyrinogen-III Synthase; Synthetase, Uroporphyrinogen III; Cosynthetase, Uroporphyrinogen III; Isomerase, Uroporphyrinogen; Synthase, Uroporphyrinogen-III; Uroporphyrinogen III Synthase; Cosynthase; Uroporphyrinogen III Cosynthetase; Uroporphyrinogen Isomerase; Hydroxymethylbilane hydro-lyase (cyclizing)
Networked: 82 relevant articles (0 outcomes, 6 trials/studies)

Bio-Agent Context: Research Results

Experts

1. de Verneuil, Hubert: 6 articles (11/2013 - 01/2008)
2. Ged, C: 6 articles (10/2012 - 03/2001)
3. de Verneuil, H: 6 articles (10/2012 - 03/2001)
4. Desnick, Robert J: 6 articles (01/2011 - 06/2002)
5. Millet, Oscar: 5 articles (11/2014 - 01/2009)
6. Ged, Cécile: 5 articles (11/2013 - 01/2008)
7. Bishop, David F: 5 articles (01/2011 - 06/2002)
8. Lalanne, Magalie: 4 articles (11/2013 - 01/2008)
9. Richard, Emmanuel: 4 articles (11/2013 - 06/2008)
10. Moreau-Gaudry, François: 4 articles (07/2012 - 01/2008)

Related Diseases

1. Erythropoietic Porphyria (Congenital Erythropoietic Porphyria)
2. Inborn Genetic Diseases (Disease, Hereditary)
3. Porphyrias
4. Hemolytic Anemia
5. Erythropoietic Protoporphyria

Related Drugs and Biologics

1. Heme (Haem)
2. Uroporphyrinogens
3. Porphyrins
4. hydroxymethylbilane
5. Uroporphyrinogen Decarboxylase (Uroporphyrinogen III Decarboxylase)
6. Iron
7. Ferrochelatase (Heme Synthetase)
8. Protoporphyrinogen Oxidase (Protox)
9. Nonsense Codon (Nonsense Mutation)
10. Complementary DNA (cDNA)

Related Therapies and Procedures

1. Bone Marrow Transplantation (Transplantation, Bone Marrow)