The Gating of Polycystin Signaling Complex

Mutations in either polycystin-2 (PC2) or polycystin-1 (PC1) proteins cause severe, potentially lethal, kidney disorders (autosomal dominant polycystic kidney disease, ADPKD) and multiple extrarenal disease phenotypes. PC2, a member of the transient receptor potential channel superfamily and PC1, an...

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Autor principal:	DELMAS,PATRICK
Lenguaje:	English
Publicado:	Sociedad de Biología de Chile 2004
Materias:	Polycystin TRP channel Sensory transduction Calcium signaling Polycystic Kidney Disease
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602004000400026
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spelling	oai:scielo:S0716-976020040004000262005-06-02The Gating of Polycystin Signaling ComplexDELMAS,PATRICK Polycystin TRP channel Sensory transduction Calcium signaling Polycystic Kidney Disease Mutations in either polycystin-2 (PC2) or polycystin-1 (PC1) proteins cause severe, potentially lethal, kidney disorders (autosomal dominant polycystic kidney disease, ADPKD) and multiple extrarenal disease phenotypes. PC2, a member of the transient receptor potential channel superfamily and PC1, an orphan membrane receptor of largely unknown function, are thought to be part of a common signalling pathway. Here, I show that co-assembly of full-length PC1 with PC2 forms an ion channel signalling complex in which PC1 regulates PC2 channel gating through a structural rearrangement of the polycystin complex (Delmas et al., 2004a). These polycystin complexes function either as a receptor-cation channel or as a G-protein-coupled receptor. Thus, PC1 acts as a prototypical membrane receptor that regulates G-proteins and plasmalemmal PC2, a bimodal mechanism that may account for the multifunctional roles of polycystin proteins in various cell types. Genetic alteration of polycystin proteins such as those occurring in kidney diseases may impede polycystin signalling, thereby providing a likely mechanistic explanation to the pathogenesis of ADPKD. Our proposed mechanism may also be paradigmatic for the function of polycystin orthologues and other polycystin-related proteins in a variety of nonrenal cell types, including sperm, muscle cells and sensory neuronsinfo:eu-repo/semantics/openAccessSociedad de Biología de ChileBiological Research v.37 n.4 20042004-01-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602004000400026en10.4067/S0716-97602004000400026
institution	Scielo Chile
collection	Scielo Chile
language	English
topic	Polycystin TRP channel Sensory transduction Calcium signaling Polycystic Kidney Disease
spellingShingle	Polycystin TRP channel Sensory transduction Calcium signaling Polycystic Kidney Disease DELMAS,PATRICK The Gating of Polycystin Signaling Complex
description	Mutations in either polycystin-2 (PC2) or polycystin-1 (PC1) proteins cause severe, potentially lethal, kidney disorders (autosomal dominant polycystic kidney disease, ADPKD) and multiple extrarenal disease phenotypes. PC2, a member of the transient receptor potential channel superfamily and PC1, an orphan membrane receptor of largely unknown function, are thought to be part of a common signalling pathway. Here, I show that co-assembly of full-length PC1 with PC2 forms an ion channel signalling complex in which PC1 regulates PC2 channel gating through a structural rearrangement of the polycystin complex (Delmas et al., 2004a). These polycystin complexes function either as a receptor-cation channel or as a G-protein-coupled receptor. Thus, PC1 acts as a prototypical membrane receptor that regulates G-proteins and plasmalemmal PC2, a bimodal mechanism that may account for the multifunctional roles of polycystin proteins in various cell types. Genetic alteration of polycystin proteins such as those occurring in kidney diseases may impede polycystin signalling, thereby providing a likely mechanistic explanation to the pathogenesis of ADPKD. Our proposed mechanism may also be paradigmatic for the function of polycystin orthologues and other polycystin-related proteins in a variety of nonrenal cell types, including sperm, muscle cells and sensory neurons
author	DELMAS,PATRICK
author_facet	DELMAS,PATRICK
author_sort	DELMAS,PATRICK
title	The Gating of Polycystin Signaling Complex
title_short	The Gating of Polycystin Signaling Complex
title_full	The Gating of Polycystin Signaling Complex
title_fullStr	The Gating of Polycystin Signaling Complex
title_full_unstemmed	The Gating of Polycystin Signaling Complex
title_sort	gating of polycystin signaling complex
publisher	Sociedad de Biología de Chile
publishDate	2004
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602004000400026
work_keys_str_mv	AT delmaspatrick thegatingofpolycystinsignalingcomplex AT delmaspatrick gatingofpolycystinsignalingcomplex
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The Gating of Polycystin Signaling Complex

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