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Erschienen in: Pediatric Nephrology 7/2004

01.07.2004 | Review

Protein reabsorption in renal proximal tubule—function and dysfunction in kidney pathophysiology

verfasst von: Erik I. Christensen, Jakub Gburek

Erschienen in: Pediatric Nephrology | Ausgabe 7/2004

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Abstract

The endocytic receptors megalin and cubilin are highly expressed in the early parts of the endocytic apparatus of the renal proximal tubule. The two receptors appear to be responsible for the tubular clearance of most proteins filtered in the glomeruli. Since cubilin is a peripheral membrane protein it has no endocytosis signaling sequence. Cubilin binds to megalin and it appears that megalin is responsible for internalization of cubilin and its ligands, in addition to internalizing its own ligands. The importance of the receptors is underscored by the proteinuria observed in megalin-deficient mice, in dogs lacking functional cubilin, and in patients with distinct mutations of the cubilin gene. In this review we focus on the role of megalin- and cubilin-mediated endocytosis in renal pathophysiology. Association between disorders characterized by tubular proteinuria, such as megaloblastic anemia type-1, Dent disease, cystinosis, and Fabry disease and the dysfunction of proximal tubular endocytosis is discussed. The correlation between the high capacity of endocytosis in the proximal tubule and progressive renal disease in overload proteinuria is considered.
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Metadaten
Titel
Protein reabsorption in renal proximal tubule—function and dysfunction in kidney pathophysiology
verfasst von
Erik I. Christensen
Jakub Gburek
Publikationsdatum
01.07.2004
Verlag
Springer-Verlag
Erschienen in
Pediatric Nephrology / Ausgabe 7/2004
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-004-1494-0

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