Porphobilinogen Synthase (delta-Aminolevulinate Dehydratase)

An enzyme that catalyzes the formation of porphobilinogen from two molecules of 5-aminolevulinic acid. EC 4.2.1.24.

Also Known As:

delta-Aminolevulinate Dehydratase; delta-Aminolevulinic Acid Dehydratase; Synthase, Porphobilinogen; ALA-Dehydrase; ALA Dehydrase; Acid Dehydratase, Aminolevulinic; Acid Dehydratase, delta-Aminolevulinic; Aminolevulinate Hydro Lyase; Dehydratase, Aminolevulinic Acid; Dehydratase, delta-Aminolevulinate; Dehydratase, delta-Aminolevulinic Acid; Hydro-Lyase, Aminolevulinate; delta Aminolevulinate Dehydratase; delta Aminolevulinic Acid Dehydratase; Aminolevulinate Hydro-Lyase; Aminolevulinic Acid Dehydratase; 5-Aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing)

Networked: 183 relevant articles (0 outcomes, 16 trials/studies)

Bio-Agent Context: Research Results

Enzymes and Coenzymes: 1
- Enzymes: 152586
  - Lyases: 637
    - Carbon-Oxygen Lyases
      - Hydro-Lyases: 266
        Porphobilinogen Synthase: 183
        Acute Hepatic Porphyria: 105

Experts

1.	Jaffe, Eileen K: 4 articles (01/2013 - 04/2006)
2.	Akagi, R: 4 articles (01/2004 - 03/2000)
3.	Sassa, S: 4 articles (01/2004 - 03/2000)
4.	de Lucca, Leidiane: 3 articles (03/2022 - 12/2016)
5.	Rocha, João Batista Teixeira: 3 articles (01/2019 - 12/2008)
6.	Hu, Howard: 3 articles (01/2016 - 08/2003)
7.	Emanuelli, Tatiana: 3 articles (10/2012 - 06/2007)
8.	Rocha, João B T: 3 articles (11/2007 - 02/2007)
9.	Doss, M O: 3 articles (01/2004 - 03/2000)
10.	Stölzel, Ulrich: 2 articles (09/2021 - 01/2019)

Related Diseases

1.	Hepatic Porphyrias (Hepatic Porphyria) 04/01/1991 - "Hereditary hepatic porphyria due to homozygous delta-aminolevulinic acid dehydratase deficiency: studies in lymphocytes and erythrocytes." 12/01/1987 - "Enzymatic defect in a child with hereditary hepatic porphyria due to homozygous delta-aminolevulinic acid dehydratase deficiency: immunochemical studies." 10/15/1979 - "New type of hepatic porphyria with porphobilinogen synthase defect and intermittent acute clinical manifestation." 01/09/1996 - "Certain of the hepatic porphyrias are classified as belonging to the acute hepatic prophyrias, namely acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP) and ALA-dehydrase deficiency. " 01/01/2023 - "Acute hepatic porphyrias include acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, and aminolevulinic acid dehydratase deficiency porphyria. "
2.	Hypothyroidism 09/01/2007 - "The aim of this study was to investigate the potential relationship between hypothyroidism and delta-aminolevulinate dehydratase (delta-ALA-D) activity in rat blood and liver. " 09/01/2007 - "Experimental hypothyroidism inhibits delta-aminolevulinate dehydratase activity in neonatal rat blood and liver." 03/01/2007 - "Delta-aminolevulinate dehydratase (delta-ALA-D) activity in diabetes and hypothyroidism." 03/01/2007 - "This study analyzed the influence of type 2 diabetes mellitus and primary hypothyroidism on the activity of the delta-aminolevulinate dehydratase (delta-ALA-D) in human blood. "
3.	Lead Poisoning (Poisoning, Lead) 12/01/2008 - "A cross-section study was conducted to explore the association between polymorphism of delta-aminolevulinic acid dehydratase (ALAD) and lead poisoning in Uygur and Han children in China. " 11/01/2017 - "To determine if SNPs located within the 3'-UTR of δ-aminolevulinic acid dehydratase (ALAD) can alter the risk of lead poisoning and the ALAD gene expression. " 08/01/2008 - "Delta -aminolevulinic acid dehydratase (ALAD) plays an important role in lead poisoning, and polymorphisms in this gene may affect the response of individuals to lead toxicity symptoms. " 07/01/2006 - "Delta aminolevulinic acid dehydratase (ALAD) plays an important role in lead poisoning. " 01/12/2001 - "Human porphobilinogen synthase (PBGS) is a main target in lead poisoning. "
4.	Porphyrias (Porphyria) 01/01/2010 - "Each of these relationships has been established for the prototype morpheein, porphobilinogen synthase, where the conformational disease is a porphyria and the drug application is in antimicrobial discovery. " 02/01/2007 - "ALAD porphyria is a rare porphyric disorder, with five documented compound heterozygous patients, and it is caused by a profound lack of porphobilinogen synthase (PBGS) activity. " 04/01/2006 - "delta-Aminolevulinate dehydratase (ALAD) porphyria: the first case in North America with two novel ALAD mutations." 05/15/2001 - "Highly heterogeneous nature of delta-aminolevulinate dehydratase (ALAD) deficiencies in ALAD porphyria." 05/01/1992 - "Cloning and expression of the defective genes from a patient with delta-aminolevulinate dehydratase porphyria."
5.	Body Weight (Weight, Body) 01/01/2016 - "As result, Pb decreased body weight, hematocrit and blood δ-aminolevulinic acid dehydratase (ALA-D) activity. " 01/01/1980 - "After a single intraperitoneal administration of lead in very small doses [1--100 micrograms Pbac/kg body weight (bw)], there was a dose-dependent, highly significant inhibition of erythrocyte D-aminolevulinic acid dehydratase (D-ALA-D) activity in mice. " 12/01/1977 - "II. Effects on delta-aminolevulinic acid dehydratase activity, hematocrit and body weight." 07/01/2000 - "Toads received one single injection with 10, 25, 50 or 100 mg/kg of body weight, equivalent to approximately 1/90-1/10 of the 120 h-LD50; seven days after the injections, the hematocrit and the blood delta-aminolevulinic acid dehydratase (ALAD) activity were measured. " 11/01/2011 - "In males but not females, feed consumption, body weight gain, packed cell volume (PCV), plasma protein concentration, and δ-aminolevulinic acid dehydratase (δ-ALAD) activity were all adversely affected by five Pb fragments. "

Related Drugs and Biologics

1.	Enzymes
2.	Aminolevulinic Acid (Levulan)
3.	Adenosine Diphosphate (ADP)
4.	Acute Hepatic Porphyria
5.	protoporphyrin IX
6.	Iron
7.	succinylacetone
8.	Acids
9.	Glutathione (Reduced Glutathione)
10.	Glucose (Dextrose)

Related Therapies and Procedures

1.	Renal Dialysis (Hemodialysis)
2.	Enzyme Replacement Therapy
3.	Kidney Transplantation
4.	Therapeutics
5.	Continuous Ambulatory Peritoneal Dialysis (CAPD)