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Uroporphyrinogen decarboxylase deficiency in experimental chronic hepatic porphyria.
Hoppe Seylers Z Physiol Chem. 1980 Aug; 361(8):1217-22.HS

Abstract

During hexachlorobenzene feeding of rats the following biochemical signs of a chronic hepatic porphyria developed: porphyrinuria with increase of uro- and hexacarboxyporphyrin, hepatic prophyrin accumulation of uro- and heptacarboxyporphyrin and a diminished activity of uroporphyrinogen decarboxylase in the liver, but nut in the red cells. During the 5.3 days of the intoxication the behaviour of metabolite constellation and enzyme activities was inverse. Hexachlorobenzene porphyria in rats is a pathobiochemical model of chronic hepatic prophyria, which in man becomes clinically manifest as porphyria cutanea tarda.

Authors

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Pub Type(s)

Journal Article

Language

eng

PubMed ID

7409755

Citation

von Tiepermann, R, et al. "Uroporphyrinogen Decarboxylase Deficiency in Experimental Chronic Hepatic Porphyria." Hoppe-Seyler's Zeitschrift Fur Physiologische Chemie, vol. 361, no. 8, 1980, pp. 1217-22.
von Tiepermann R, Koss G, Doss M. Uroporphyrinogen decarboxylase deficiency in experimental chronic hepatic porphyria. Hoppe-Seyler's Z Physiol Chem. 1980;361(8):1217-22.
von Tiepermann, R., Koss, G., & Doss, M. (1980). Uroporphyrinogen decarboxylase deficiency in experimental chronic hepatic porphyria. Hoppe-Seyler's Zeitschrift Fur Physiologische Chemie, 361(8), 1217-22.
von Tiepermann R, Koss G, Doss M. Uroporphyrinogen Decarboxylase Deficiency in Experimental Chronic Hepatic Porphyria. Hoppe-Seyler's Z Physiol Chem. 1980;361(8):1217-22. PubMed PMID: 7409755.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Uroporphyrinogen decarboxylase deficiency in experimental chronic hepatic porphyria. AU - von Tiepermann,R, AU - Koss,G, AU - Doss,M, PY - 1980/8/1/pubmed PY - 1980/8/1/medline PY - 1980/8/1/entrez SP - 1217 EP - 22 JF - Hoppe-Seyler's Zeitschrift fur physiologische Chemie JO - Hoppe-Seyler's Z. Physiol. Chem. VL - 361 IS - 8 N2 - During hexachlorobenzene feeding of rats the following biochemical signs of a chronic hepatic porphyria developed: porphyrinuria with increase of uro- and hexacarboxyporphyrin, hepatic prophyrin accumulation of uro- and heptacarboxyporphyrin and a diminished activity of uroporphyrinogen decarboxylase in the liver, but nut in the red cells. During the 5.3 days of the intoxication the behaviour of metabolite constellation and enzyme activities was inverse. Hexachlorobenzene porphyria in rats is a pathobiochemical model of chronic hepatic prophyria, which in man becomes clinically manifest as porphyria cutanea tarda. SN - 0018-4888 UR - https://www.unboundmedicine.com/medline/citation/7409755/Uroporphyrinogen_decarboxylase_deficiency_in_experimental_chronic_hepatic_porphyria_ L2 - https://medlineplus.gov/porphyria.html DB - PRIME DP - Unbound Medicine ER -