Variegate Porphyria

2020 ◽  
Author(s):  
Keyword(s):  
Variegate Porphyria

Protoporphyrinogen oxidase, porphobilinogen deaminase and uroporphyrinogen decarboxylase in variegate porphyria

1985 ◽  
Vol 13 (1) ◽  
pp. 203-204 ◽  
Author(s):  
P. N. MEISSNER ◽  
E. D. STURROCK ◽  
M. R. MOORE ◽  
P. B. DISLER ◽  
D. L. MAEDER
Keyword(s):  
Porphobilinogen Deaminase ◽  
Uroporphyrinogen Decarboxylase ◽  
Protoporphyrinogen Oxidase ◽  
Variegate Porphyria

Variegate porphyria

1980 ◽  
Vol 2 (1) ◽  
pp. 36-43 ◽  
Author(s):  
Timothy J. Corey ◽  
Vincent A. DeLeo ◽  
Herbert Christianson ◽  
Maureen B. Poh-Fitzpatrick
Keyword(s):  
Variegate Porphyria

Homozygous variegate porphyria presenting with developmental and language delay in childhood

2013 ◽  
Vol 38 (7) ◽  
pp. 737-740 ◽  
Author(s):  
V. A. E. Pinder ◽  
S. T. Holden ◽  
C. Deshpande ◽  
A. Siddiqui ◽  
J. E. Mellerio ◽  
...  
Keyword(s):  
Language Delay ◽  
Variegate Porphyria

scholarly journals Variegate porphyria induces plasma and neutrophil oxidative stress: effects of dietary supplementation with vitamins E and C

2009 ◽  
Vol 103 (1) ◽  
pp. 69-76 ◽  
Author(s):  
Miguel D. Ferrer ◽  
Pedro Tauler ◽  
Antoni Sureda ◽  
Clara Palacín ◽  
Josep A. Tur ◽  
...  
Keyword(s):  
Vitamin E ◽  
Vitamin C ◽  
Oxidative Damage ◽  
Antioxidant Activities ◽  
Dietary Supplementation ◽  
Antioxidant Defenses ◽  
Ros Production ◽  
Double Blind ◽  
Variegate Porphyria ◽  
Vitamins E And C

Our aim was to analyse the influence of variegate porphyria (VP) on the antioxidant defenses and markers of oxidative damage and inflammation in plasma and neutrophils and the effects of dietary supplementation with vitamins E and C on these parameters in plasma, neutrophils and erythrocytes. Twelve women affected by VP and twelve pair-matched healthy control women participated in a double-blind crossover study. Each participant took 50 mg/d of vitamin E and 150 mg/d of vitamin C, or a placebo, for 6 months, by consuming an almond-based beverage as the vehicle. Women affected by VP presented higher C-reactive protein and malondialdehyde (MDA) circulating levels. Plasma antioxidant defenses were not different between porphyric and control women. Neutrophils from VP women presented decreased catalase (CAT) and glutathione reductase (GR) activities together with increased protein carbonyl levels. Reactive oxygen species (ROS) production from stimulated neutrophils was also higher in porphyric women than their controls. Dietary supplementation was effective in increasing α-tocopherol levels in neutrophils and in reducing MDA levels in plasma. Erythrocyte CAT and GR activities were enhanced by the enriched beverage only in the control subjects. In conclusion, women affected by VP present a situation of inflammation, plasma oxidative damage and neutrophils more primed to the oxidative burst, with decreased antioxidant activities and increased ROS production capabilities and protein oxidative damage. Dietary supplementation with vitamin E (50 mg/d) and vitamin C (150 mg/d) for 6 months decreased plasma oxidative damage and enhanced the erythrocyte activities of CAT and GR.

Effects of haem arginate on variegate porphyria

1990 ◽  
Vol 123 (3) ◽  
pp. 381-387 ◽  
Author(s):  
K. TIMONEN ◽  
P. MUSTAJOKI ◽  
R. TENHUNEN ◽  
J. LAUHARANTA
Keyword(s):  
Variegate Porphyria

Brainstem dysfunction in variegate porphyria

2012 ◽  
Vol 46 (3) ◽  
pp. 426-433 ◽  
Author(s):  
Gonzalo Barraza ◽  
Tereza Serranova ◽  
Carmen Herrero ◽  
Jordi Casanova-mollá ◽  
Jordi To-figueras ◽  
...  
Keyword(s):  
Variegate Porphyria ◽  
Brainstem Dysfunction

Antioxidants restore protoporphyrinogen oxidase in variegate porphyria patients

2013 ◽  
Vol 43 (7) ◽  
pp. 668-678 ◽  
Author(s):  
Miguel D. Ferrer ◽  
Pedro Tauler ◽  
Antoni Sureda ◽  
Clara Palacín ◽  
Josep A. Tur ◽  
...  
Keyword(s):  
Protoporphyrinogen Oxidase ◽  
Variegate Porphyria

Linkage between the variegate porphyria (VP) and the alpha-1-antitrypsin (PI) genes on human chromosome 14

1988 ◽  
Vol 79 (3) ◽  
pp. 289-290 ◽  
Author(s):  
S. Bissbort ◽  
H. W. Hitzeroth ◽  
D. P. du Wentzel ◽  
C. W. Van den Berg ◽  
H. Senff ◽  
...  
Keyword(s):  
Human Chromosome ◽  
Chromosome 14 ◽  
Variegate Porphyria ◽  
Alpha 1 Antitrypsin

Variegate porphyria in a 46-year-old patient taking sibutramine for weight loss

2010 ◽  
Vol 11 (4) ◽  
pp. 329-331
Author(s):  
M. Reiser ◽  
S. Eickmann ◽  
T. Haverkamp ◽  
U. Finckh
Keyword(s):  
Weight Loss ◽  
Variegate Porphyria

scholarly journals Homozygous Variegate Porphyria: Identification of Mutations on Both Alleles of the Protoporphyrinogen Oxidase Gene in a Severely Affected Proband

1998 ◽  
Vol 110 (4) ◽  
pp. 452-455 ◽  
Author(s):  
Jorge Frank ◽  
John McGrath ◽  
HaMut Lam ◽  
Robert M. Graham ◽  
John L.M. Hawk ◽  
...  
Keyword(s):  
Oxidase Gene ◽  
Protoporphyrinogen Oxidase ◽  
Variegate Porphyria
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