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Hydroxymethylbilane Synthase (Porphobilinogen Deaminase)

An enzyme that catalyzes the tetrapolymerization of the monopyrrole PORPHOBILINOGEN into the hydroxymethylbilane preuroporphyrinogen (UROPORPHYRINOGENS) in several discrete steps. It is the third enzyme in the 8-enzyme biosynthetic pathway of HEME. In humans, deficiency in this enzyme encoded by HMBS (or PBGD) gene results in a form of neurological porphyria (PORPHYRIA, ACUTE INTERMITTENT). This enzyme was formerly listed as EC 4.3.1.8
Also Known As:
Porphobilinogen Deaminase; Hydroxymethylbilane Synthetase; Pre-uroporphyrinogen Synthetase; Preuroporphyrinogen Synthetase; Ammonia-Lyase, Porphobilinogen; Deaminase, Porphobilinogen; Porphobilinogen Ammonia Lyase; Pre uroporphyrinogen Synthetase; Synthase, Hydroxymethylbilane; Synthase, Uroporphyrinogen I; Synthetase, Hydroxymethylbilane; Synthetase, Pre-uroporphyrinogen; Synthetase, Preuroporphyrinogen; Porphobilinogen Ammonia-Lyase; Uroporphyrinogen I Synthase; Porphobilinogen ammonia-lyase (polymerizing)
Networked: 272 relevant articles (3 outcomes, 22 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1. Harper, Pauline: 8 articles (09/2015 - 08/2003)
2. Fontanellas, Antonio: 7 articles (09/2015 - 03/2010)
3. Sampedro, Ana: 7 articles (09/2015 - 03/2010)
4. Mauleón, Itsaso: 6 articles (09/2015 - 03/2010)
5. Schneider-Yin, Xiaoye: 6 articles (03/2015 - 01/2002)
6. Minder, Elisabeth I: 6 articles (03/2015 - 01/2002)
7. Unzu, Carmen: 6 articles (12/2013 - 03/2010)
8. Prieto, Jesús: 5 articles (07/2013 - 03/2010)
9. Puy, H: 5 articles (09/2009 - 07/2000)
10. Deybach, Jean-Charles: 4 articles (09/2015 - 01/2002)

Related Diseases

1. Acute Intermittent Porphyria (Porphyria, Acute)
2. Glioma (Gliomas)
3. Neoplasms (Cancer)
4. Adenocarcinoma
5. Schizophrenia (Dementia Praecox)

Related Drugs and Biologics

1. Heme (Haem)
2. protoporphyrin IX
3. Aminolevulinic Acid (Levulan)
4. Phenobarbital (Luminal)
5. Butyrates (Butyrate)
6. Oxidoreductases
7. TATA-Box Binding Protein (TATA-Binding Protein)
8. Succinate Dehydrogenase (Fumarate Reductase)
9. Enzymes
10. Protoporphyrinogen Oxidase (Protox)

Related Therapies and Procedures

1. Enzyme Replacement Therapy
2. Ovariectomy (Oophorectomy)
3. Photochemotherapy (Photodynamic Therapy)
4. Nephrectomy