The Activity of Erythrocyte Porphobilinogen Deaminase in Familial and Sporadic Forms of Porphyria cutanea tarda

2015 ◽  
pp. 116-122
Author(s):  
P. D. Siersema ◽  
F. W. M. de Rooij ◽  
A. Edixhoven-Bosdijk ◽  
J. H. P. Wilson
Keyword(s):  
Porphyria Cutanea Tarda ◽  
Porphobilinogen Deaminase

Erythrocyte porphobilinogen deaminase activity in porphyria cutanea tarda

1990 ◽  
Vol 36 (10) ◽  
pp. 1779-1783 ◽  
Author(s):  
P D Siersema ◽  
F W de Rooij ◽  
A Edixhoven-Bosdijk ◽  
J H Wilson
Keyword(s):  
Porphyria Cutanea Tarda ◽  
Polyclonal Antibodies ◽  
Inverse Correlation ◽  
Absolute Amount ◽  
Porphobilinogen Deaminase ◽  
Uroporphyrinogen Decarboxylase ◽  
Asymptomatic Group ◽  
Asymptomatic Patients ◽  
Minor Degree ◽  
A Minor

Abstract Porphyria cutanea tarda (PCT) results from a metabolic block in heme synthesis at the level of uroporphyrinogen decarboxylase. We measured the activity of one of the enzymes preceding it in the heme biosynthetic pathway, porphobilinogen deaminase (PBGD; EC 4.3.1.8), in erythrocytes of 47 patients with symptomatic or asymptomatic familial or sporadic PCT. PBGD activity was significantly increased in all four PCT groups, compared with controls. To study the mechanism of this increased PBGD activity, we determined, using polyclonal antibodies, the amount of immuno-detectable PBGD per 100 units of PBGD activity (Ig PBGD/100 U) and the total amount of immuno-detectable PBGD (Ig PBGD) in erythrocytes from all 47 patients and from controls. In both familial and sporadic PCT, Ig PBGD/100 U was decreased compared with that in controls (P less than 0.05). Especially in asymptomatic patients of the familial PCT group there was an inverse correlation between increasing PBGD activity and Ig PBGD/100 U (r = -0.90). In familial PCT, and to a minor degree in sporadic PCT, an increase in PBGD activity was accompanied by an increased Ig PBGD, compared with controls (familial PCT: P less than 0.001, sporadic PCT: P less than 0.05). In familial and sporadic PCT an increase in erythrocyte PBGD activity can, at least partly, be explained by a diminished degradation of PBGD. In familial PCT, in the symptomatic group more than in the asymptomatic group, and to a minor degree in sporadic PCT, there is in addition an increase in the absolute amount of PBGD.

The blister of porphyria cutanea tarda: A Scanning and Transmission Electron Microscopic study

1986 ◽  
Vol 44 ◽  
pp. 334-335
Author(s):  
Veronika Burmeister ◽  
R. Swaminathan
Keyword(s):  
Electron Microscopy ◽  
Transmission Electron Microscopy ◽  
Basement Membrane ◽  
Middle Age ◽  
Porphyria Cutanea Tarda ◽  
Minor Trauma ◽  
Electron Microscopic ◽  
Transmission Electron Microscopic Study ◽  
Transmission Electron ◽  
Transmission Electron Microscopic

Porphyria cutanea tarda (PCT) is a disorder of porphyrin metabolism which occurs most often during middle age. The disease is characterized by excessive production of uroporphyrin which causes photosensitivity and skin eruptions on hands and arms, due to minor trauma and exposure to sunlight. The pathology of the blister is well known, being subepidermal with epidermodermal separation, it is not always absolutely clear, whether the basal lamina is attached to the epidermis or the dermis. The purpose of our investigation was to study the attachment of the basement membrane in the blister by comparing scanning with transmission electron microscopy.

Abstract #14: Oral Contraceptive-Induced Porphyria Cutanea Tarda

2003 ◽  
Vol 9 ◽  
pp. 5-6
Author(s):  
Manju Chandran ◽  
Roopa Sathyaprakash ◽  
R. Jeffrey Chang
Keyword(s):  
Oral Contraceptive ◽  
Porphyria Cutanea Tarda
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