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

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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.

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Acknowledgements

We are grateful to Julia Klippert for critical reading of the manuscript. Work in the authors’ laboratories was supported by grants SFB665 (to TEW) and CH1838/1-1 (to AC) from the Deutsche Forschungsgemeinschaft.

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  1. Max-Delbrueck Center for Molecular Medicine, Robert-Roessle-Str. 10, 13125, Berlin, Germany

    Thomas E. Willnow & Annabel Christ

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  1. Thomas E. Willnow
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This article is part of the special issue on Functional Anatomy of the Kidney in Health and Disease in Pflügers Archiv – European Journal of Physiology

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Willnow, T.E., Christ, A. Endocytic receptor LRP2/megalin—of holoprosencephaly and renal Fanconi syndrome. Pflugers Arch - Eur J Physiol 469, 907–916 (2017). https://doi.org/10.1007/s00424-017-1992-0

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  • DOI: https://doi.org/10.1007/s00424-017-1992-0

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