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Porphyria cutanea tarda is thought to result from an inherited deficiency of uroporphyrinogen decarboxylase (EC 4.1.1.37) in some patients. Present methods for determining uroporphyrinogen decarboxylase activity are time consuming, so we examined the pattern of porphyrins formed from porphobilinogen by hemolysates as a possible marker for hereditary porphyria cutanea tarda. After the hemolysates are incubated with porphobilinogen, the porphyrins are converted to their methyl esters and examined by liquid chromatography, with fluorometric detection. The porphyrinic patients examined, and some of their relatives, showed a characteristic pattern of porphyrin production, with high uroporphyrin/coproporphyrin and (uroporphyrin + heptacarboxylic porphyrins)/coproporphyrin ratios, at least partly ascribable to increased uroporphyrinogen I synthetase (EC 4.2.1.8) activity in patients' hemolysates, and also to a relative deficiency of uroporphyrinogen decarboxylase. Examination of the pattern of porphyrins produced from porphobilinogen by hemolysates is a suitable technique for detecting asymptomatic individuals with porphyria cutanea tarda.
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
January 1984,
Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia,
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
August 1985,
The Australasian journal of dermatology,
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
September 1973,
Dermatologische Monatschrift,
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
October 1990,
Clinical chemistry,
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
October 1988,
Journal of medical genetics,
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
November 1969,
Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete,
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
June 1975,
The British journal of dermatology,
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
January 1991,
Current problems in dermatology,
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
January 1996,
Dermatology (Basel, Switzerland),
M A Alleman, and
J H Wilson, and
J W van den Berg, and
A Edixhoven-Bosdijk, and
L M van Gastel-Quist
April 1970,
Deutsche medizinische Wochenschrift (1946),
