Abstract
Sialic acid (SA) is mainly bound to glycoconjugates and is a component of glomerular membranes. Free SA is the most abundant negatively charged carbohydrate in urine. Salla Disease (SD) is a lysosomal storage disorder due to impaired egress of free SA from lysosomes. SD patients have 5-10 times elevated free SA in their urine and plasma.
We determined the renal clearance of SA in controls and SD patients over a broad range of filtered loads, using an HPLC method. All patients had a normal creatinine clearance or glomerular filtration rate (GFR). The SA clearance approximated the GFR in all subjects, regardless of filtered load; hence, the fractional excretion of SA was approximately 100%. These results suggest that free SA is filtered by the kidneys and not reabsorbed, which contrasts markedly with the renal handling of glucose, which is efficiently reabsorbed at plasma concentrations 1000 times that of free SA.
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Renlund, M., Seppälä, R., Tietze, F. et al. 30 RENAL HANDLING OF SIALIC ACID (SA). Pediatr Res 24, 265 (1988). https://doi.org/10.1203/00006450-198808000-00056
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DOI: https://doi.org/10.1203/00006450-198808000-00056
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