Pentosuria
Encyclopedia
Pentosuria is a condition where xylulose
Xylulose
Xylulose is a ketopentose, a monosaccharide containing five carbon atoms, and including a ketone functional group. It has the chemical formula 5105. In nature, it occurs in both the L- and D-enantiomers.- Pathology :...

 (a pentose
Pentose
A pentose is a monosaccharide with five carbon atoms. Pentoses are organized into two groups. Aldopentoses have an aldehyde functional group at position 1...

) presents in the urine
Urine
Urine is a typically sterile liquid by-product of the body that is secreted by the kidneys through a process called urination and excreted through the urethra. Cellular metabolism generates numerous by-products, many rich in nitrogen, that require elimination from the bloodstream...

 in unusually high concentrations.

It is associated with a deficiency of L-xylulose reductase
L-xylulose reductase
L-xylulose reductase is an enzyme responsible for the metabolism of xylulose, converting it into xylitol....

.

L-xylulose is a reducing sugar so it may give false diagnosis of diabetes as it is found in high concentrations in urine

It was characterized as an inborn error of metabolism in 1908.
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