Porphyria cutanea tarda (PCT) is characterized biochemically by excessive hepatic synthesis and urinary excretion of uroporphyrin I. Clinical evidence has implicated iron in the pathogenesis of PCT. The synthesis of the normally occurring isomer of uroporphyrin, namely uroporphyrin III, from porphobilinogen (PBG) requires two enzymes; uroporphyrinogen I synthetase and uroporphyrinogen III cosynthetase (COSYN). In the absence of COSYN only uroporphyrinogen I is formed.
J. P. Kushner, G. R. Lee, S. Nacht
Title and authors | Publication | Year |
---|---|---|
Uroporphyria in the Cyp1a2-/- mouse
JD Phillips, JP Kushner, HA Bergonia, MR Franklin |
Blood cells, molecules & diseases | 2011 |
Hepatic Iron Quantitation and Its Relationship with Disease Measures and Histologically Assessed Iron Content
S Sajjad, M Garcia, A Malik, DH Thiel |
Digestive Diseases and Sciences | 2007 |
Hepatic iron quantitation and its relationship with disease measures and histologically assessed iron content.
Sajjad S, Garcia M, Malik A, Van Thiel DH |
Digestive Diseases and Sciences | 2007 |
The Porphyrin Handbook
PM Shoolingin-Jordan |
The Porphyrin Handbook | 2003 |
Comprehensive Natural Products Chemistry
LJ Marnett, DC Goodwin, SW Rowlinson, AS Kalgutkar, LM Landino |
Comprehensive Natural Products Chemistry | 1999 |
Normal and Abnormal Heme Biosynthesis. 2. 1 Synthesis and Metabolism of Type-III Pentacarboxylic Porphyrinogens: Further Experimental Evidence for the Enzymic Clockwise Decarboxylation of Uroporphyrinogen-III
TD Lash, UN Mani, EA Lyons, P Thientanavanich, MA Jones |
The Journal of Organic Chemistry | 1998 |
Tissue-Specific Regulation of Iron Metabolism and Heme Synthesis: Distinct Control Mechanisms in Erythroid Cells
P Ponka |
Blood | 1997 |
Purification and properties of the uroporphyrinogen decarboxylase from Rhodobacter sphaeroides
RM Jones, PM Jordan |
Biochemical Journal | 1993 |
Ergebnisse der Inneren Medizin und Kinderheilkunde/Advances in Internal Medicine and Pediatrics
M Brandis, A Fanconi, P Frick, K Kochsiek, EO Riecken |
1992 | |
Mechanism of iron potentiation of hepatic uroporphyria: Studies in cultured chick embryo liver cells
HL Bonkovsky |
Hepatology | 1989 |
8 The clinical manifestations of chronic iron overload
CE Halliday, JW Halliday, LW Powell |
Baillière's Clinical Haematology | 1989 |
Association between Alcoholism and Increased Hepatic Iron Stores
MG Irving, JW Halliday, LW Powell |
Alcoholism, clinical and experimental research | 1988 |
Porphyria Cutanea Tarda and Porphyria-Like Bullous Dermatoses Associated with Chronic Hemodialysis
MB Poh-Fitzpatrick |
Seminars in Dialysis | 1988 |
Hepatic heme synthesis in a new model of experimental hemochromatosis: Studies in rats fed finely divided elemental iron
HL Bonkovsky, JF Healey, B Lincoln, BR Bacon, DF Bishop, GH Elder |
Hepatology | 1987 |
Mechanistic studies of the inhibition of hepatic uroporphyrinogen decarboxylase in C57BL/10 mice by iron-hexachlorobenzene synergism
AG Smith, JE Francis, SJ Kay, JB Greig, FP Stewart |
Biochemical Journal | 1986 |
Porphyria cutanea tarda, or the uroporphyrinogen decarboxylase deficiency diseases
GD Sweeney |
Clinical Biochemistry | 1986 |
Porphyria Cutanea Tarda
ME Grossman, MB Poh-Fitzpatrick |
Dermatologic Clinics | 1986 |
Stimulation of 5-aminolevulinic acid metabolism by uroporphyryinogen I in rat liver homogenate
A Shivji, M Bernstein |
Clinical Biochemistry | 1985 |
Dual mechanism of inhibition of rat liver uroporphyrinogen decarboxylase activity by ferrous iron: Its potential role in the genesis of porphyria cutanea tarda
SK Mukerji, NR Pimstone, M Burns |
Gastroenterology | 1984 |
The Porphyrias
SW Eubanks, JW Patterson, DL May, JL Aeling |
International Journal of Dermatology | 1983 |
Hepatic pathology in porphyria cutanea tarda
JH Lefkowitch, ME Grossman |
Liver | 1983 |
Developmental aspects of hepatic heme biosynthetic capability and hematotoxicity—II. Studies on uroporphyrinogen decarboxylase
JS Woods, RM Kardish |
Biochemical Pharmacology | 1983 |
Effects of Phlebotomy on Urinary Porphyrin Pattern and Liver Histology in Patients with Porphyria Cutanea Tarda
CD Padova, L Marchesi, T Cainelli, G Gori, SA Podenzani, P Rovagnati, M Rizzardini, L Cantoni |
The American Journal of the Medical Sciences | 1983 |
Rat hepatic uroporphyrinogen III cosynthase: Purification, properties, and inhibition by metal ions
RP Clement, M Kohashi, WN Piper |
Archives of Biochemistry and Biophysics | 1982 |
Pathology of a Black African Population
C Isaacson |
1982 | |
The Effect of Ethanol on Heme and Porphyrin Synthesis
JW Ostroff |
Alcoholism, clinical and experimental research | 1981 |
Treatment of the Porphyrias: Mechanisms of Action
DR Bickers |
Journal of Investigative Dermatology | 1981 |
Conversion of 5-aminolaevulinate into haem by liver homogenates. Comparison of rat and chick embryo
JF Healey, HL Bonkowsky, PR Sinclair, JF Sinclair |
Biochemical Journal | 1981 |
Iron and the liver. Acute and long-term effects of iron-loading on hepatic haem metabolism
HL Bonkowsky, JF Healey, PR Sinclair, JF Sinclair, JS Pomeroy |
Biochemical Journal | 1981 |
Studies on the action of porphyrinogenic trace metals on the activity of hepatic uroporphyrinogen decarboxylase
JS Woods, R Kardish, BA Fowler |
Biochemical and Biophysical Research Communications | 1981 |
Hemochromatosis-newer concepts: Diagnosis and management
ML Bassett, JW Halliday, LW Powell |
Disease-a-Month | 1980 |
Porphyria Cutanea Tarda: Diagnosis and Management
ME Grossman, MB Poh-Fitzpatrick |
Medical Clinics of North America | 1980 |
PORPHYRIA CUTANEA TARDA
JE Muhlbauer, MA Pathak |
International Journal of Dermatology | 1979 |
Skin diseases in Zambia
AV Ratnam, K Jayaraju |
British Journal of Dermatology | 1979 |
Hepatic heme metabolism and its control
HL Bonkowsky, PR Sinclair, JF Sinclair |
The Yale journal of biology and medicine | 1979 |
Porphyria Cutanea Tarda in Two Patients Treated with Hemodialysis for Chronic Renal Failure
MB Poh-Fitzpatrick, N Bellet, VA DeLeo, ME Grossman, DR Bickers |
New England Journal of Medicine | 1978 |
Decreased Activity of Hepatic Uroporphyrinogen Decarboxylase in Sporadic Porphyria Cutanea Tarda
GH Elder, GB Lee, JA Tovey |
New England Journal of Medicine | 1978 |
Porphyria Cutanea Tarda in Three Generations of a Single Family
AV Benedetto, JP Kushner, JS Taylor |
New England Journal of Medicine | 1978 |
Heme and Hemoproteins
F Matteis, WN Aldridge |
1978 | |
ENZYME DEFICIENCIES IN THE PORPHYRIAS
S Kramer, DM Becker, JD Viljoen |
British Journal of Haematology | 1977 |
Porphyrin biosynthesis from porphobilinogen by duck blood hemolysate
RB Frydman, B Frydman, G Feinstein |
Molecular and Cellular Biochemistry | 1977 |
Haemophilia, hepatitis and porphyria
J Burnett, J Lamon, J Levin |
British Journal of Dermatology | 1977 |
Hepatic porphyrias current concepts
JR Walsh |
Postgraduate Medicine | 1977 |
Porphyrin biosynthesis from prophobilinogen by duck blood hemolysate.
Frydman RB, Frydman B, Feinstein G |
Molecular and Cellular Biochemistry | 1977 |
An inherited enzymatic defect in porphyria cutanea tarda: decreased uroporphyrinogen decarboxylase activity
JP Kushner, AJ Barbuto, GR Lee |
Journal of Clinical Investigation | 1976 |
The Hepatic Porphyrias
JR Bloomer |
Gastroenterology | 1976 |
The role of iron in the pathogenesis of porphyria cutanea tarda. II. Inhibition of uroporphyrinogen decarboxylase
JP Kushner, DP Steinmuller, GR Lee |
Journal of Clinical Investigation | 1975 |
Experimental hepatic porphyria induced by polychlorinated biphenyls
JA Goldstein, P Hickman, DL Jue |
Toxicology and Applied Pharmacology | 1974 |
Uroporphyrin formation induced by chlorinated hydrocarbons (lindane, polychlorinated biphenyls, tetrachlorodibenzo-p-dioxin). Requirements for endogenous iron, protein synthesis and drug-metabolizing activity
PR Sinclair, S Granick |
Biochemical and Biophysical Research Communications | 1974 |
Iron Storage Disorders of the Liver
ND Grace, LW Powell |
Gastroenterology | 1974 |
Porphyria cutanea tarda
H Ippen |
Archiv f�r Klinische und Experimentelle Dermatologie | 1959 |