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Endocytic receptor LRP2/megalin-of holoprosencephaly and renal Fanconi syndrome

Item Type:Review
Title:Endocytic receptor LRP2/megalin-of holoprosencephaly and renal Fanconi syndrome
Creators Name:Willnow, T.E. and Christ, A.
Abstract:Megalin (or LRP2) is an endocytic receptor that plays a central role in embryonic development and adult tissue homeostasis. Loss of this receptor in congenital or acquired diseases results in multiple organ dysfunctions, including forebrain malformation (holoprosencephaly) and renal reabsorption defects (renal Fanconi syndrome). Here, we describe current concepts of the mode of receptor action that include co-receptors and a repertoire of different ligands, and we discuss how these interactions govern functional integrity of the kidney and the brain, and cause disease when defective.
Keywords:Endocytosis, Forebrain Development, Proteinuria, Proximal Convoluted Tubules, Sonic Hedgehog, Vitamin D, Animals
Source:Pflugers Archiv
ISSN:0031-6768
Publisher:Springer
Volume:469
Number:7-8
Page Range:907-916
Date:August 2017
Official Publication:https://doi.org/10.1007/s00424-017-1992-0
PubMed:View item in PubMed

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