LP15945-6
Uroporphyrinogen decarboxylase
Active
Descriptions
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. Source: National Library of Medicine, MeSH 2006
Uroprophyrinogen Decarboxylase (Upg D) deficiency of Upg D activity causes an accumulation of uroporphyrins and hepta-, hexa-, and pentacarboxyl porphyrins in the skin, subcutaneous tissues, and liver, often resulting in the most common form of porphyria, porphyria cutanea tarda (PCT). Rarely, hepatoerythropoietic porphyria (HEP) results. Photosensitivity and skin fragility occurs in both PCT and HEP. HEP presents in early childhood, while PCT usually presents in adults. PCT type I and type III are associated with normal red cell Upg D activity, while PCT type II will have decreased red cell Upg D activity. About 20% of porphphyria cutanea tartada patients are PCT type II. Information from Tietz Clinical Guide to Laboratory Tests, A.Wu, 4th ed, 2006. Source: Regenstrief Institute
Basic Part Properties
- Part Display Name
- Uroporphyrinogen decarboxylase
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2000-05-04
- Construct for LOINC Short Name
- URO-D
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- CodeSystem lookup
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Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| zh-CN | Chinese (China) | 尿卟啉原脱羧酶 Synonyms: Upg D; |
| fr-CA | French (Canada) | Uroporphyrinogène décarboxylase |
| et-EE | Estonian (Estonia) | Uroporfürinogeeni dekarboksülaas |
| es-ES | Spanish (Spain) | Uroporfirinógeno decarboxilasa |
| it-IT | Italian (Italy) | Uroporfirinogeno decarbossilasi |
| tr-TR | Turkish (Turkey) | Üroporfirinojen dekarboksilaz |
| ru-RU | Russian (Russian Federation) | Уропорфириноген декарбоксилаза Synonyms: Уропорфириноген-декарбоксилаза |
| nl-NL | Dutch (Netherlands) | uroporfyrinogeendecarboxylase |
| fr-BE | French (Belgium) | Uroporphyrinogène décarboxylase |
| pl-PL | Polish (Poland) | Dekarboksylaza uroporfirynogenu |
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