porphyria cutanea tarda/جلوتاثيون

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Effects of vitamin E administration on the hemorheological status and redox homeostasis of patients with porphyria cutanea tarda treated with phlebotomy.

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BACKGROUND Conflicting results were reported about the efficacy of vitamin E (E) treatment in porphyria cutanea tarda (PCT). We conducted a study in PCT patients to investigate whether E treatment has any additional beneficial effects compared with phlebotomy (P) treatment alone on rheological and

Longitudinal study of a mouse model of familial porphyria cutanea tarda.

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Most rodent models of porphyria cutanea tarda (PCT) share in common the administration of iron and agents that induce transcription of cytochrome P450s. Dissection of changes related to porphyrin accumulation required generation of a genetic model free from exogenous precipitants. Mice heterozygous

[Evaluation of the effects of reduced glutathione in subjects with acquired and congenital changes in heme biosynthesis].

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In order to evaluate the possible therapeutical effects of reduced glutathione in subjects affected by acquired or congenital heme biosynthesis alterations, two groups of subjects have been considered: the first, of 5 subjects with abnormal lead absorption, the second of 10 patients suffering from

Enhanced regional expression of glutathione S-transferase P1-1 with colocalized AP-1 and CYP 1A2 induction in chlorobenzene-induced porphyria.

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A distinct, nonfocal expression pattern was observed for glutathione S-transferase P1-1 (rGSTP1-1) in rats exposed to either hexachloro-(HCB) or pentachlorobenzene (PeCB). The nonfocal expression was localized to the centrilobular region with the most intense staining nearest the central vein. A

Study of erythrocyte delta-aminolevulinic acid dehydratase activity in porphyria cutanea tarda.

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The erythrocyte delta-aminolevulinic acid dehydratase activity was studied in porphyria cutanea tarda patients, compared to healthy controls, in an attempt to resolve the contradictions in the relevant literature data. In an in vitro experimental system, a study was also made of how the erythrocyte

CYP1A21F and GSTM1 alleles are associated with susceptibility to porphyria cutanea tarda*.

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Porphyria cutanea tarda (PCT) is a cutaneous porphyria with sporadic (type 1) and familial (type 2) subtypes, both resulting from decreased hepatic uroporphyrinogen decarboxylase (UROD) activity. Environmental and genetic factors are involved in the development of PCT, and genetic variants in the

Expression and characterization of six clinically relevant uroporphyrinogen decarboxylase gene mutations.

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The functional consequence of six uroporphyrinogen decarboxylase (UROD) gene mutations found in Danish patients with familial porphyria cutanea tarda was investigated. Wild-type UROD and the 6 mutants (3 missense, 1 nonsense and 2 frameshift mutants) were cloned and expressed using the prokaryotic

Volatile anaesthetics induce biochemical alterations in the heme pathway in a B-lymphocyte cell line established from hepatoerythropoietic porphyria patients (LBHEP) and in mice inoculated with LBHEP cells.

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Hepatoerythropoietic porphyria (HEP) is the homozygous form of Porphyria Cutanea Tarda (PCT), characterized by an accumulation of porphyrins due to uroporphyrinogen decarboxylase deficiency. Fluorinated volatile anaesthetics are often used to produce general anaesthesia. Anaesthesia has certainly

Protective effect of S-adenosyl-L-methionine in hepatic uroporphyria. Evaluation in an experimental model.

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The potential use of S-adenosyl-L-methionine (SAMe) as therapy for human porphyria cutanea tarda was investigated in an experimental model of hepatic porphyria--that is, chronic treatment of female rats with 0.2% hexachlorobenzene (HCB) in the diet. Administration of SAMe (25 mg/kg subcutaneously

Hepatic heme synthesis in a new model of experimental hemochromatosis: studies in rats fed finely divided elemental iron.

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Rats fed chow containing finely divided elemental iron (from carbonyl-iron) develop hepatic iron overload resembling human hereditary hemochromatosis in that deposition of iron is primarily in periportal hepatocytes and with hepatic iron concentrations sufficiently high to be associated in the human

How does hexachlorobenzene treatment affect liver uroporphyrinogen decarboxylase?

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The aims of the present work were: (1) to investigate whether the strong decrease of liver uroporphyrinogen decarboxylase (UroD) activity observed in experimental porphyria cutanea tarda is due to alteration of the enzymatic protein and (2) to improve the knowledge about the normal liver enzyme.

Circulating pro- and antioxidant factors in iron and porphyrin metabolism disorders.

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BACKGROUND Porphyria cutanea tarda and haemochromatosis are taken to be spontaneous human models of oxidative cellular damage, with an increased risk of fibrosis and cancer evolution. OBJECTIVE To define the relative pro-oxidant roles of porphyrin and iron, in their different molecular forms, and

[Clinical review of pseudoporphyria].

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Pseudoporphyria is a photosensitive bullous disease, which resembles porphyria cutanea tarda. Normal porphyrin levels in urine, stool and blood define pseudoporphyria. Pseudoporphyria is associated with chronic renal failure, haemodialysis, a variety of drugs (e.g. naproxen, nabumetone, furosemide,

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porphyria cutanea tarda/جلوتاثيون

Effects of vitamin E administration on the hemorheological status and redox homeostasis of patients with porphyria cutanea tarda treated with phlebotomy.

Longitudinal study of a mouse model of familial porphyria cutanea tarda.

[Evaluation of the effects of reduced glutathione in subjects with acquired and congenital changes in heme biosynthesis].

Enhanced regional expression of glutathione S-transferase P1-1 with colocalized AP-1 and CYP 1A2 induction in chlorobenzene-induced porphyria.

Study of erythrocyte delta-aminolevulinic acid dehydratase activity in porphyria cutanea tarda.

CYP1A2*1F and GSTM1 alleles are associated with susceptibility to porphyria cutanea tarda.

Expression and characterization of six clinically relevant uroporphyrinogen decarboxylase gene mutations.

Volatile anaesthetics induce biochemical alterations in the heme pathway in a B-lymphocyte cell line established from hepatoerythropoietic porphyria patients (LBHEP) and in mice inoculated with LBHEP cells.

Protective effect of S-adenosyl-L-methionine in hepatic uroporphyria. Evaluation in an experimental model.

Hepatic heme synthesis in a new model of experimental hemochromatosis: studies in rats fed finely divided elemental iron.

How does hexachlorobenzene treatment affect liver uroporphyrinogen decarboxylase?

Circulating pro- and antioxidant factors in iron and porphyrin metabolism disorders.

[Clinical review of pseudoporphyria].

قاعدة بيانات الأعشاب الطبية الأكثر اكتمالا التي يدعمها العلم

CYP1A21F and GSTM1 alleles are associated with susceptibility to porphyria cutanea tarda*.