Erythrocyte uroporphyrinogen decarboxylase activity and therapeutic phlebotomy in porphyria cutanea tarda

F. Koszo, M. Morvay, A. Dobozy, K. Boda

Research output: Contribution to journalArticle

Abstract

On the basis of the uroporphyrinogen decarboxylase (UD) activity in the erythrocytes, and the family history of the disease, different types of porphyria cutanea tarda (PCT) can be distinguished. In some cases, however, the distinction may involve some uncertainty (overlapping of subgroups). The question arises of whether the current erythrocyte UD activities in the different types of PCT are determined merely genetically. The erythrocyte UD activities in 72 unrelated patients with different forms of PCT (62 with type I PCT and 10 with type II PCT), in different stages of the disease, were measured in order to test whether the activity exhibits any change during the long period of recovery. In both types the activities were faintly but significantly increased, from 94.9% (in PCT I) or 54.3% (in PCT II) up to 98.4% or 56.1% respectively. In both types the lower activity in the untreated condition can be attributed to a combination of several factors, including oxidative damage to UD, which results in a minor additional inhibition of the genetically determined enzyme activities. Copyright (C) 2000 John Wiley and Sons, Ltd.

Original languageEnglish
Pages (from-to)736-738
Number of pages3
JournalJournal of Porphyrins and Phthalocyanines
Volume4
Issue number8
DOIs
Publication statusPublished - 2000

Fingerprint

Uroporphyrinogen Decarboxylase
Enzyme inhibition
Enzyme activity
Recovery

Keywords

  • Phlebotomy
  • Porphyria cutanea tarda
  • Uroporphyrinogen decarboxylase

ASJC Scopus subject areas

  • Chemistry(all)

Cite this

Erythrocyte uroporphyrinogen decarboxylase activity and therapeutic phlebotomy in porphyria cutanea tarda. / Koszo, F.; Morvay, M.; Dobozy, A.; Boda, K.

In: Journal of Porphyrins and Phthalocyanines, Vol. 4, No. 8, 2000, p. 736-738.

Research output: Contribution to journalArticle

@article{09aa3734178b4f18ae143dc4ec1ddeca,
title = "Erythrocyte uroporphyrinogen decarboxylase activity and therapeutic phlebotomy in porphyria cutanea tarda",
abstract = "On the basis of the uroporphyrinogen decarboxylase (UD) activity in the erythrocytes, and the family history of the disease, different types of porphyria cutanea tarda (PCT) can be distinguished. In some cases, however, the distinction may involve some uncertainty (overlapping of subgroups). The question arises of whether the current erythrocyte UD activities in the different types of PCT are determined merely genetically. The erythrocyte UD activities in 72 unrelated patients with different forms of PCT (62 with type I PCT and 10 with type II PCT), in different stages of the disease, were measured in order to test whether the activity exhibits any change during the long period of recovery. In both types the activities were faintly but significantly increased, from 94.9{\%} (in PCT I) or 54.3{\%} (in PCT II) up to 98.4{\%} or 56.1{\%} respectively. In both types the lower activity in the untreated condition can be attributed to a combination of several factors, including oxidative damage to UD, which results in a minor additional inhibition of the genetically determined enzyme activities. Copyright (C) 2000 John Wiley and Sons, Ltd.",
keywords = "Phlebotomy, Porphyria cutanea tarda, Uroporphyrinogen decarboxylase",
author = "F. Koszo and M. Morvay and A. Dobozy and K. Boda",
year = "2000",
doi = "10.1002/1099-1409(200012)4:8<736::AID-JPP289>3.0.CO;2-J",
language = "English",
volume = "4",
pages = "736--738",
journal = "Journal of Porphyrins and Phthalocyanines",
issn = "1088-4246",
publisher = "Society of Porphyrins and Phthalocyanines (SPP)",
number = "8",

}

TY - JOUR

T1 - Erythrocyte uroporphyrinogen decarboxylase activity and therapeutic phlebotomy in porphyria cutanea tarda

AU - Koszo, F.

AU - Morvay, M.

AU - Dobozy, A.

AU - Boda, K.

PY - 2000

Y1 - 2000

N2 - On the basis of the uroporphyrinogen decarboxylase (UD) activity in the erythrocytes, and the family history of the disease, different types of porphyria cutanea tarda (PCT) can be distinguished. In some cases, however, the distinction may involve some uncertainty (overlapping of subgroups). The question arises of whether the current erythrocyte UD activities in the different types of PCT are determined merely genetically. The erythrocyte UD activities in 72 unrelated patients with different forms of PCT (62 with type I PCT and 10 with type II PCT), in different stages of the disease, were measured in order to test whether the activity exhibits any change during the long period of recovery. In both types the activities were faintly but significantly increased, from 94.9% (in PCT I) or 54.3% (in PCT II) up to 98.4% or 56.1% respectively. In both types the lower activity in the untreated condition can be attributed to a combination of several factors, including oxidative damage to UD, which results in a minor additional inhibition of the genetically determined enzyme activities. Copyright (C) 2000 John Wiley and Sons, Ltd.

AB - On the basis of the uroporphyrinogen decarboxylase (UD) activity in the erythrocytes, and the family history of the disease, different types of porphyria cutanea tarda (PCT) can be distinguished. In some cases, however, the distinction may involve some uncertainty (overlapping of subgroups). The question arises of whether the current erythrocyte UD activities in the different types of PCT are determined merely genetically. The erythrocyte UD activities in 72 unrelated patients with different forms of PCT (62 with type I PCT and 10 with type II PCT), in different stages of the disease, were measured in order to test whether the activity exhibits any change during the long period of recovery. In both types the activities were faintly but significantly increased, from 94.9% (in PCT I) or 54.3% (in PCT II) up to 98.4% or 56.1% respectively. In both types the lower activity in the untreated condition can be attributed to a combination of several factors, including oxidative damage to UD, which results in a minor additional inhibition of the genetically determined enzyme activities. Copyright (C) 2000 John Wiley and Sons, Ltd.

KW - Phlebotomy

KW - Porphyria cutanea tarda

KW - Uroporphyrinogen decarboxylase

UR - http://www.scopus.com/inward/record.url?scp=0034537520&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034537520&partnerID=8YFLogxK

U2 - 10.1002/1099-1409(200012)4:8<736::AID-JPP289>3.0.CO;2-J

DO - 10.1002/1099-1409(200012)4:8<736::AID-JPP289>3.0.CO;2-J

M3 - Article

VL - 4

SP - 736

EP - 738

JO - Journal of Porphyrins and Phthalocyanines

JF - Journal of Porphyrins and Phthalocyanines

SN - 1088-4246

IS - 8

ER -