Structured Summary
Abstract
An autosomal dominant porphyria that is due to a deficiency of protoporphyrinogen oxidase (EC 1.3.3.4) in the LIVER, the seventh enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include both neurological symptoms and cutaneous lesions. Patients excrete increased levels of porphyrin precursors, COPROPORPHYRINS and protoporphyrinogen.
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Classification
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Synonyms
8 entry terms
- Porphyria Variegata
- Porphyria Variegate
- Porphyria, South African Type
- Porphyria, Variegate
- Ppox Deficiency
- Protoporphyrinogen Oxidase Deficiency
- Deficiency, Ppox
- Ppox Deficiencies
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Aspects Covered
35 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, congenital, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
MeSH Record
History Note
2005; use PORPHYRIA, HEPATIC 1993-2004
MeSH Record
Previous Indexing
- Porphyria (1964-2004)
- Porphyria, Hepatic (1993-2004)
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AMA Style
References
- National Library of Medicine. Variegate Porphyria. Medical Subject Headings (MeSH). 2026. Unique ID D046350. http://id.nlm.nih.gov/mesh/2026/D046350
- Variegate Porphyria. In: Wikipedia. https://en.wikipedia.org/wiki/Variegate_porphyria
- Variegate Porphyria. In: Wikidata. https://www.wikidata.org/wiki/Q275385