Open AccessLysosomal enzymuria is a feature of hereditary Fanconi syndrome and is related to elevated CI-mannose-6-P-receptor excretion
Author(s) -
Anthony G.W. Norden,
Sharon. C. Gardner,
William van’t Hoff,
Robert J. Unwin
Publication year - 2008
Publication title -
nephrology dialysis transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.654
H-Index - 168
eISSN - 1460-2385
pISSN - 0931-0509
DOI - 10.1093/ndt/gfm898
Subject(s) - excretion , endocrinology , medicine , cathepsin , cathepsin d , cathepsin b , mucolipidosis , microbiology and biotechnology , enzyme , biology , biochemistry
Lysosomal enzymuria is usually considered to be a non-specific marker of renal injury, but little is known about lysosomal enzyme excretion in renal proximal tubular cell disorders such as the renal Fanconi syndrome (FS). We examined excretion of two lysosomal enzymes and the cation-independent mannose-6-phosphate receptor (CI-MPR) in patients with inherited FS.
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