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Porphyria, Variegate MeSH Descriptor Data 2023


MeSH Heading
Porphyria, Variegate
Tree Number(s)
C06.552.830.625
C16.320.850.742.625
C17.800.827.742.625
C18.452.811.400.625
Unique ID
D046350
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D046350
Scope Note
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.
Entry Term(s)
Porphyria Variegata
Porphyria Variegate
Porphyria, South African Type
Ppox Deficiency
Protoporphyrinogen Oxidase Deficiency
Variegate Porphyria
Previous Indexing
Porphyria (1964-2004)
Porphyria, Hepatic (1993-2004)
Public MeSH Note
2005; see PORPHYRIA, HEPATIC 1993-2004
History Note
2005; use PORPHYRIA, HEPATIC 1993-2004
Date Established
2005/01/01
Date of Entry
2004/07/15
Revision Date
2020/02/28
Porphyria, Variegate Preferred
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