The tandem endocytic receptors megalin and cubilin are important proteins in renal pathology.
The molecular mechanisms controlling proximal tubule reabsorption of proteins have been much elucidated in recent years. Megalin and cubilin constitute two important endocytic receptor proteins involved in this process. Although structurally very different the two receptor proteins interact to mediate the reabsorption of a large number of filtered proteins, including carrier proteins important for transport and cellular uptake of several vitamins, lipids and other nutrients. Dysfunction of either protein results in tubular proteinuria and is associated with specific changes in vitamin metabolism due to the defective proximal tubular reabsorption of carrier proteins. Additional focus on the two receptors is attracted by the possible pathogenic role of excessive tubular protein uptake during conditions of increased filtration of proteins, and by recent findings implicating members of the low density lipoprotein-receptor family, which includes megalin, in the transduction of signals by association with cytoplasmic proteins.
Verroust
Pierre J
PJ
Institut National de la Santé et de la Recherche Médicale U538, Centre Hôpitale Universitaire, St. Antoine, Paris, France.
Birn
Henrik
H
Nielsen
Rikke
R
Kozyraki
Renata
R
Christensen
Erik Ilsø
EI
D000818:Animals; D006801:Humans; D007674:Kidney Diseases / Q000378:metabolism* / Q000473:pathology*; D026561:Low Density Lipoprotein Receptor-Related Protein-2 / Q000378:metabolism*; D011956:Receptors, Cell Surface / Q000378:metabolism*; D022161:Transport Vesicles / Q000378:metabolism*
D026561:Low Density Lipoprotein Receptor-Related Protein-2; D011956:Receptors, Cell Surface; C029486:intrinsic factor-cobalamin receptor