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NDT Advance Access originally published online on December 8, 2007
Nephrology Dialysis Transplantation 2008 23(3):813-815; doi:10.1093/ndt/gfm720
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


A spoonful of sugar helps the proteinuria go down?*

Peter Topham1,2, Jonathan Barratt1,2 and John Feehally1,2

1 Department of Infection, Immunity and Inflammation, University of Leicester and John Walls Renal Unit, Leicester, UK 2 University Hospitals of Leicester NHS Trust, Leicester, UK

Dr Peter Topham, Department of Infection, Immunity & Inflammation, John Walls Renal Unit, Leicester General Hospital, Gwendolen Road, Leicester, LE5 4PW, UK. Tel: +44-0116-258-4122; Fax: +44-0116-258-4764; E-mail: peter.topham@uhl-tr.nhs.uk

Keywords: proteinuria; glomerulonephritis; glycosylation; sialic acid; HIBM

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This paper describes kidney defects in knock-in mice homozygous for the M712T mutation in the gene encoding for uridine diphospho-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE/MNK). In humans, this genetic defect causes hereditary inclusion body myopathy (HIBM), an autosomal recessive neuromuscular disorder characterized by adult onset, slowly progressive muscle weakness and atrophy. GNE/MNK is ubiquitously expressed and catalyses the first two rate-limiting steps in the biosynthesis of 5-N-acetylneuraminic acid (sialic acid). HIBM is thought to result from hyposialylation of muscle glycoproteins. The aim of the study was to generate a model of HIBM, in which the hypothesis that supplementation with free sialic acid could ameliorate the muscle disease could be tested. Unexpectedly, . . . [Full Text of this Article]



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