These porphyrins are reddish pigments that accumulate in the liver and are disseminated in plasma to other organs. (medscape.com)
Substrates for the deficient enzyme, which are porphyrinogens (reduced porphyrins), accumulate in the liver, are oxidized to porphyrins, transported to the skin, and cause photosensitivity. (bmj.com)
Intermediates3
Porphyrinogens are intermediates in the biosynthesis of porphyrins, cofactors with a porphine core which are found in many enzymes and proteins including myoglobin, hemoglobin, cytochromes, and chlorophylls. (wikipedia.org)
Subsequent biosynthetic intermediates en route to porphyrins are deeply colored and often phytotoxic. (wikipedia.org)
Hepatic tumors producing excess porphyrins are rare causes of porphyria cutanea tarda-like disorders. (medscape.com)
Porphyrin by-products of the pathway exit the hepatocytes, are distributed throughout the body in blood plasma, mediate photooxidative chemical reactions causing skin lesions, and yield the abnormal excretory porphyrin profiles that characterize porphyria cutanea tarda. (medscape.com)
Other common features of porphyria cutanea tarda include hypertrichosis, sclerodermalike plaques that may develop dystrophic calcification, and excretion of discolored urine that resembles port wine or tea, which is due to the presence of porphyrin pigments. (medscape.com)
de Viale, L.C.S.M. 'Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias' (1980) Clinica Chimica Acta. (uba.ar)
Substrates1
these excess substrates then undergo iron-facilitated spontaneous oxidization to photoactive porphyrins. (medscape.com)
Heme5
When hepatic UROD activity falls below the critical threshold, porphyrin by-products of the heme biosynthetic pathway with 4-8 carboxyl group substituents are overproduced. (medscape.com)
Place an Order The Iron and Heme Core provides analysis of metals, precursor porphyrins and heme. (utah.edu)
The core specializes in the UPLC quantification of heme and porphyrin levels, and the measurement of activity of enzymes responsible for heme biosynthesis. (utah.edu)
Heme/porphyrin/metal analysis was performed at the Iron and Heme Core facility at the University of Utah, supported in part by a grant from the NIH National Institute of Diabetes and Digestive and Kidney Diseases, Grant number U54DK110858. (utah.edu)
The Escherichia coli Protein YfeX Functions as a Porphyrinogen Oxidase, Not a Heme Dechelatase. (porphyria.org)
Urine1
Diagnosis is established by finding substantial increases in porphyrins in urine or plasma and excluding other blistering cutaneous porphyrias. (bmj.com)
Side chains2
Meso-substituted porphyrinogens with eight non-hydrogen side chains are also called calix[4]pyrroles. (wikipedia.org)
Porphyrins with four methyl and four propionic acid side chains attached to the pyrrole rings. (jefferson.edu)
Core2
In biochemistry a porphyrinogen is a member of a class of naturally occurring compounds with a tetrapyrrole core, a macrocycle of four pyrrole rings connected by four methylene bridges. (wikipedia.org)
Oxidation turns the central hexahydroporphine core into a porphine core, yielding the desired porphyrin. (wikipedia.org)
Skin1
Photoexcited porphyrins in the skin mediate oxidative damage to biomolecular targets, causing cutaneous lesions. (medscape.com)
Rings1
Porphyrins differ from porphyrinogens by having the four pyrrole rings linked by methine bridges =CH− instead of methylene bridges −CH2−, and by lacking the hydrogen atom in two of the four amine −NH− groups, turning them into imines =N−. In the biosynthesis of porphyrins, the parent porphyrinogen is dehydrogenated by protoporphyrinogen oxidase. (wikipedia.org)
Excess porphyrins3
Hepatic tumors producing excess porphyrins are rare causes of porphyria cutanea tarda-like disorders. (medscape.com)
The interaction of excess porphyrins in the skin and light radiation causes photoxidative damage of biomolecular targets that is manifested as mechanical fragility and blistering that may result in severe scarring. (medscape.com)
Mercury exposure causes oxidative damage to the kidney, resulting in numerous biochemical changes, including the excretion of excess porphyrins in the urine (porphyrinuria). (nih.gov)
Heme synthesis3
Erythropoietic porphyria (EP) is a rare inborn error of porphyrin-heme synthesis inherited that is as an autosomal recessive trait. (medscape.com)
The neurotoxins in these conditions are porphyrin precursors (delta-aminolevulinic acid [ALA], porphobilinogen [PBG]) and porphyrinogen substrates in heme synthesis, whose levels are elevated (see the image below). (medscape.com)
Porphobilinogen, a colorless and chemically similar (tetrapyrroles) substance, is a solute found in the urine of patients with abnormal porphyrin metabolism (heme synthesis). (basicmedicalkey.com)
Photoactive3
these excess substrates then undergo iron-facilitated spontaneous oxidization to photoactive porphyrins. (medscape.com)
These are converted by spontaneous oxidation into their corresponding photoactive porphyrins leading to photodamage. (nih.gov)
The photoactive nature of porphyrin molecules results in the bright pink fluorescence of these pigments in urine, teeth, and bones under Wood light illumination. (medscape.com)
Oxidation4
Oxidation turns the central hexahydroporphine core into a porphine core, yielding the desired porphyrin. (wikipedia.org)
Hg(II)-induced porphyrinuria may occur, in part, by the previously reported oxidation of reduced porphyrins (porphyrinogens) by a GSH/Hg(II) complex and H2O2. (nih.gov)
To further elucidate the mechanism(s) of porphyrinogen oxidation by GSH, Hg(II), and H2O2, we compared the ability of several thiol compounds and peroxides to substitute for GSH or H2O2, respectively, in the oxidation of uroporphyrinogen (urogen). (nih.gov)
These findings suggest that mercury-thiol complexes possess redox activity in biological systems, which promotes the oxidation of porphyrinogens and possibly other biomolecules. (nih.gov)
There is resultant accumulation and hyperexcretion of porphyrinogens of the isomer I variety. (nih.gov)
Accumulation of porphyrins results in haemolysis and extensive photosensitivity. (nih.gov)
The inheritance of 2 mutant alleles for the gene encoding the enzyme uroporphyrinogen III synthase leads to accumulation of porphyrins of the isomer I type that are biologically useless but cause cutaneous photosensitivity characterized by blisters, erosions, and scarring of light-exposed skin. (medscape.com)
Pyrrole rings2
In biochemistry a porphyrinogen is a member of a class of naturally occurring compounds with a tetrapyrrole core, a macrocycle of four pyrrole rings connected by four methylene bridges. (wikipedia.org)
Porphyrins differ from porphyrinogens by having the four pyrrole rings linked by methine bridges =CH− instead of methylene bridges −CH2−, and by lacking the hydrogen atom in two of the four amine −NH− groups, turning them into imines =N−. In the biosynthesis of porphyrins, the parent porphyrinogen is dehydrogenated by protoporphyrinogen oxidase. (wikipedia.org)
The accumulated isomer I porphyrinogens are spontaneously oxidized to their corresponding porphyrins, which are water-soluble photosensitizers with a reddish hue. (medscape.com)
Chlorophylls2
Porphyrinogens are intermediates in the biosynthesis of porphyrins, cofactors with a porphine core which are found in many enzymes and proteins including myoglobin, hemoglobin, cytochromes, and chlorophylls. (wikipedia.org)
Pyrroles are components of more complex macrocycles, including the porphyrinogens and products derived therefrom, including porphyrins of heme , the chlorins , bacteriochlorins, and chlorophylls . (cloudfront.net)
Water soluble1
Porphyrins with high carboxyl group numbers are water soluble and excreted primarily by renal mechanisms. (medscape.com)
Deposition2
bones and teeth are stained red by the deposition of porphyrin pigment. (medscape.com)
Porphyrin deposition may lead to corneal ulcers and scarring, reddish-brown discoloration of the teeth (erythrodontia), and bone loss and/or expansion of the bone marrow. (nih.gov)
Abnormal1
Porphyrin by-products of the pathway exit the hepatocytes, are distributed throughout the body in blood plasma, mediate photooxidative chemical reactions causing skin lesions, and yield the abnormal excretory porphyrin profiles that characterize porphyria cutanea tarda. (medscape.com)
Biosynthetic1
When hepatic UROD activity falls below the critical threshold, porphyrin by-products of the heme biosynthetic pathway with 4-8 carboxyl group substituents are overproduced. (medscape.com)
A reaction intermediate of HemN activity was isolated by HPLC analysis and identified as monovinyltripropionic acid porphyrin by mass spectrometry. (uni-hannover.de)
Products1
These products resist dehydrogenation of the outer ring better than the natural porphyrinogens. (wikipedia.org)