Nature and Significance of Pentosuria in Neuromuscular Disease

Tower, D. B.; Peters, E. L.; Pogorelskin, M. A.

doi:10.1212/wnl.6.1.37

Cited by 19 publications

(5 citation statements)

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“…There have been several reports of a ribosuria associated with muscular dystrophy (52) and neoplastic disease (53). Whether this ribosuria is a primary defect in these diseases, or is merely a consequence of tissue breakdown has not been established definitely.…”

Section: Discussionmentioning

confidence: 99%

The Metabolism of D-Ribose in Man

Segal¹,

Foley²

1958

J. Clin. Invest.

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Section: Discussionmentioning

confidence: 99%

The Metabolism of D-Ribose in Man

Segal¹,

Foley²

1958

J. Clin. Invest.

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“…Several pentoses have been identified in normal urine: the aldopentoses xylose, arabinose and ribose (3,4) and the ketopentoses D-ribulose and L-xylulose (5). Large quantities of the latter sugar are excreted in the disease essential pentosuria (6, 7), which appears to be due to a defect in the further conversion of L-xylulOse to the sugar alcohol xylitol (8).…”

mentioning

confidence: 99%

The Metabolic Fate of C14 Labeled Pentoses in Man

Segal¹,

Foley²

1959

J. Clin. Invest.

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The role of pentoses in intermediary metabolism has received much attention within the past few years. The phosphate esters of D-ribose, D-ribulose and D-xylulose have been identified as key intermediates in the pentose phosphate pathway of glucose metabolism in both plant and mammalian tissue (1). More recently, another alternate pathway of glucose dissimilation, the uronic acid pathway, has been described in which L-Xylulose, xylitol and D-xylulose are intermediates (2).Several pentoses have been identified in normal urine: the aldopentoses xylose, arabinose and ribose (3, 4) and the ketopentoses D-ribulose and L-xylulose (5). Large quantities of the latter sugar are excreted in the disease essential pentosuria (6, 7), which appears to be due to a defect in the further conversion of L-xylulOse to the sugar alcohol xylitol (8). In addition to L-xylulOse Touster and Harwell (9) have also reported the isolation of L-arabitol from the urine of pentosuric subjects.Because of increasing awareness of the biological importance of the pentoses, certain aspects of pentose metabolism in man have been investigated in this laboratory. Previous reports have described the physiological disposition of large quantities of infused pentoses (10), the effect of insulin on blood levels of infused pentoses (11) as well as studies of the metabolism of D-ribose (12). This paper reports some observations on the fate of C14 labeled D-xylOse, D-lyxose, D-arabinose and L-arabinose administered in trace quantities intravenously to normal human subjects. Some of the previously published data (12) on C14 ribose metabolism are included for purposes of comparison. METHODSFive experiments were performed in four normal male volunteers aged 18 to 21. Each subject had been on a 250 Gm. carbohydrate diet for at least three days and was fasted overnight prior to study.Five /Ac. of each sugar dissolved in 250 ml. of normal saline were infused over a 15 minute period. Expired air samples were collected for 4 minutes at 15 to 60 minute intervals for 6 hours. Urine samples were collected at hourly intervals for 6 hours and pooled thereafter till 24 hours had elapsed from the start of the experiment. A urine sample was obtained at the twentyfifth hour to determine if C" was still being excreted. Urine was preserved by freezing at -20' C. Blood wvas collected at 20 minute intervals.Methods for the collection and C' counting of expired CO,, for preparation and radioactive assay of blood samples, and C' counting and chromatography of labeled compounds in urine have previously been described (12).The C" pentoses D-xylose, D-lyxose, D-arabinose, Larabinose and D-ribose (Figure 1) with specific activity of 0.67, 1.65, 1.65, 1.11, 2.6 and 2.01 ,lc. per mg., respec-H C=O H-C-OH

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“…Normal human urine regularly contains measurable quantities of the aldopentoses xylose, arabinose and ribose (1,2), and of the ketopentoses, D-ribulose and L-xylulose (2-4) in addition to small quantities of glucose, fructose, fucose, galactose, sucrose, lactose, mannoheptulose and sedoheptulose (1, 2, 5). Patients with essential pentosuria excrete gram quantities of the ketopentose, L-xylulose, independent of diet (6)(7)(8).…”

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confidence: 99%

“…Ingestion of foods high in pentosan content, especially fruits and grains, is associated with a type of pentosuria called alimentary pentosuria (9). The pentose excreted reflects that ingested, generally xylose or arabinose (1). In addition, there are poorly defined pentosurias thought to be toxic or drug-induced (10,11) or associated with neurological disease (1).…”

mentioning

confidence: 99%