Structured Summary
Abstract
An enzyme that catalyzes the decarboxylation of UROPORPHYRINOGEN III to coproporphyrinogen III by the conversion of four acetate groups to four methyl groups. It is the fifth enzyme in the 8-enzyme biosynthetic pathway of HEME. Several forms of cutaneous PORPHYRIAS are results of this enzyme deficiency as in PORPHYRIA CUTANEA TARDA; and HEPATOERYTHROPOIETIC PORPHYRIA.
MeSH Record
Classification
Broader headings
Related Concepts
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MeSH Record
Synonyms
6 entry terms
- Uroporphyrinogen Carboxy-Lyase
- Uroporphyrinogen III Decarboxylase
- Carboxy-Lyase, Uroporphyrinogen
- Decarboxylase, Uroporphyrinogen
- Decarboxylase, Uroporphyrinogen III
- Uroporphyrinogen Carboxy Lyase
MeSH Record
Aspects Covered
29 allowable subheadings
Indexed with the subheadings administration & dosage, adverse effects, analysis, antagonists & inhibitors, biosynthesis, blood, cerebrospinal fluid, chemical synthesis, chemistry, classification, deficiency, drug effects, economics, genetics, history, immunology, isolation & purification, metabolism, pharmacokinetics, pharmacology, physiology, poisoning, radiation effects, standards, supply & distribution, therapeutic use, toxicity, ultrastructure, urine.
MeSH Record
Indexing Annotation
/defic permitted but consider also PORPHYRIA CUTANEA TARDA and HEPATOERYTHROPOIETIC PORPHYRIA
MeSH Record
History Note
91(76); was see under CARBOXY-LYASES 1976-90
MeSH Record
Previous Indexing
- Carboxy-Lyases (1974-1975)
- Porphyrinogens (1975)
- Porphyrins (1974)
MeSH Hierarchy
Tree Number
AMA Style
References
- National Library of Medicine. Uroporphyrinogen Decarboxylase. Medical Subject Headings (MeSH). 2026. Unique ID D014575. http://id.nlm.nih.gov/mesh/2026/D014575
- Uroporphyrinogen Decarboxylase. In: Wikidata. https://www.wikidata.org/wiki/Q24769641