Separation of porphyrin isomers by high-performance liquid chromatography
C.K. Lim,J M Rideout,D J Wright +2 more
TLDR
A reversed-phase gradient elution system is described, adaptable for isocratic and stepwise separation of individual groups of isomers, which is also suitable for preparative isolation of pure porphyrins.Abstract:
A reversed-phase gradient elution system is described for the simultaneous separation of the type I and type III isomers of 8-, 7-, 6-, 5- and 4-carboxylated porphyrins and isocoproporphyrins. The method, adaptable for isocratic and stepwise separation of individual groups of isomers, is also suitable for preparative isolation of pure porphyrins. The analyses of porphyrin isomers in the urine and faeces of porphyric patients are examples of applications.read more
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Journal ArticleDOI
Urine and faecal porphyrin profiles by reversed-phase high-performance liquid chromatography in the porphyrias
C.K. Lim,T.J. Peters +1 more
TL;DR: The method is simple and reproducible and has been applied to the analysis of porphyrins in urine and faeces from patients with acute intermittent, variegate, hereditary copro, congenital erythropoietic, erythrohepatic proto and symptomatic porphyrias.
Journal ArticleDOI
Reduction of Uroporphyrinogen Decarboxylase by Antisense RNA Expression Affects Activities of Other Enzymes Involved in Tetrapyrrole Biosynthesis and Leads to Light-Dependent Necrosis.
Hans-Peter Mock,Bernhard Grimm +1 more
TL;DR: It is concluded that photooxidation of accumulating uroporphyrin(ogen) leads to the generation of oxygen species, which destabilizes other enzymes in the porphyrIn metabolic pathway, which resembles the induction of cell death observed during pathogenesis and air pollution.
Journal ArticleDOI
Acute intermittent porphyria: studies of the severe homozygous dominant disease provides insights into the neurologic attacks in acute porphyrias.
Constanza Solis,Martínez-Bermejo A,Thomas P. Naidich,Walter E. Kaufmann,Kenneth H. Astrin,David F. Bishop,Robert J. Desnick,Robert J. Desnick +7 more
TL;DR: It is suggested that porphyrin precursor toxicity is primarily responsible for the acute neurologic attacks in heterozygous AIP and other porphyrias and suggest that the neurologic manifestations of a severely affected infant with HD-AIP result from porphyrsine precursor toxicity rather than heme deficiency.
Journal ArticleDOI
High-performance liquid chromatography of porphyrins
TL;DR: The emphasis is on high-performance liquid chromatography and its aspplications in the quantitative analysis of porphyrins in blood, urine and faeces and qualitative porphyrsin profiles in normal subjects and in the porphyrias.
Journal ArticleDOI
Co-Inheritance of Mutations in the Uroporphyrinogen Decarboxylase and Hemochromatosis Genes Accelerates the Onset of Porphyria Cutanea Tarda
Jennifer J. Brady,Helen A. Jackson,Andrew G. Roberts,Rhian R. Morgan,Sharon D. Whatley,Gareth Lloyd Rowlands,Cindy M. Darby,T. Emma Shudell,Rosemarie Watson,Janice Paiker,Mark Worwood,George H. Elder +11 more
TL;DR: Analysis of the frequencies of hemochromatosis genotypes in each type of porphyria cutanea tarda indicated that C282Y homozygosity is an important susceptibility factor in both types but suggested that heterozygosity for this mutation has much less effect on the development of the disease.
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Urine and faecal porphyrin profiles by reversed-phase high-performance liquid chromatography in the porphyrias
C.K. Lim,T.J. Peters +1 more