Development and Implications of Experimental Renal Porphyria

Schwartz, Shepard; Stephenson, Betty; Sarkar, Debapriya; Freyholtz, H.; Runge, Walter

doi:10.1159/000398772

Cited by 6 publications

(7 citation statements)

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“…According toRimington (20) "the porphyrins, once formed, do not appear to be reducible in vivo, and, therefore, become external to the metabolic stream". In vivo oxidation of uroporphyrinogen III to biosynthetically inactive porphyrin may be the basic defect in porphyria cutanea tarda (12). The mechanism and the exact place of oxidation is not clear.…”

Section: Resultsmentioning

confidence: 99%

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Porphyrinogens in Urine in Various Types of Porphyrias

Martásek¹,

Jirsa²,

Kordač³

1982

CCLM

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Porphyrinogens were determined in very fresh, diluted morning urine in two types of hepatic porphyria, using a simple and very quick spectrophotometric method. It was shown that acidification by itself is insufficient for the complete conversion of colourless porphyrinogens into porphyrins in photometric determination of porphyrins in diluted urine. A correlation was shown between percentual content of porphyrinogens and the sum of pentacarboxyporphyrin and coproporphyrin. A difference was found between the excretion of porphyrinogens in morning fresh urine in porphyria cutanea tarda (average porphyrinogen fraction 22.5%, SD 10.3% of total porphyrins) and in acute intermittent porphyria (average porphyrinogen fraction 77.1 %, SD 9.3% of total porphyrins). The method is at the same time suitable for the detection of urobilinoids in urine. Oxidation of the urine in acute intermittent porphyria is recommended before adsorption on talc and subsequent thin j layer chromatography, because porphyrinogens are not adsorbed on talc.

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Section: Resultsmentioning

confidence: 99%

“…Schwartz et al (9) used iodine äs an oxidation agent; H 2 0 2 (10,11) and light are also used. Recently chloranil was used (12) for the oxidation of porphyrinogens into porphyrins for fluorometric determination of total porphyrins and porphyrinogens in diluted urine (l 3).…”

Section: Introductionmentioning

confidence: 99%

Porphyrinogens in Urine in Various Types of Porphyrias

Martásek¹,

Jirsa²,

Kordač³

1982

CCLM

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“…In addition to the normal kidney syn thesizing and secreting into the urine significant quanti ties of coproporphyrin III, ALA synthetase and ferrochelatase activity has also been demonstrated in the kid ney [83,84]. Schwartzet al [85] have also emphasized the important and independent role of renal haem metabo lism. Plasma coproporphyrin levels are reduced and red cell protoporphyrin levels elevated in renal failure, and this disturbance of porphyrin metabolism is reversed by renal transplantation [86].…”

Section: Porphyrins and Renal Diseasementioning

confidence: 99%

Aluminium-Induced Anaemia in Haemodialysis Patients

McGonigle

Parsons

1985

Nephron

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“…Schwartz and co-workers have long emphasized the im portant and independent role of renal heme metabolism. Amongst other things, they have shown that radioactively labelled ALA produced 4 times as much labelled heme in renal tissues than in hepatic tissues of normal dogs [9]: that ferrochelatase activity was greater in the kidney cortex than in the liver of normal hens and hamsters, although the opposite was true for ALA dehydratase [10], and that the activity of these enzymes is generally higher in the renal cortex than in the medulla of normal hamsters [10], Recent ly, a series of investigations involving humans and rats with both normal and abnormal porphyrin metabolism has been carried out in our laboratories to establish whether or not the kidney plays a major role in the synthesis of normal and excess levels of urinary porphyrins and pre cursors.…”

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confidence: 99%