Polycystic kidney disease: novel insights into polycystin function.
ADPKD
CDCA
cation channel
cilia
lumen diameter control
polycystin
Journal
Trends in molecular medicine
ISSN: 1471-499X
Titre abrégé: Trends Mol Med
Pays: England
ID NLM: 100966035
Informations de publication
Date de publication:
04 2023
04 2023
Historique:
received:
24
07
2022
revised:
18
01
2023
accepted:
24
01
2023
pubmed:
23
2
2023
medline:
21
3
2023
entrez:
22
2
2023
Statut:
ppublish
Résumé
Autosomal dominant polycystic kidney disease (ADPKD) is a life-threatening monogenic disease caused by mutations in PKD1 and PKD2 that encode polycystin 1 (PC1) and polycystin 2 (PC2). PC1/2 localize to cilia of renal epithelial cells, and their function is believed to embody an inhibitory activity that suppresses the cilia-dependent cyst activation (CDCA) signal. Consequently, PC deficiency results in activation of CDCA and stimulates cyst growth. Recently, re-expression of PCs in established cysts has been shown to reverse PKD. Thus, the mode of action of PCs resembles a 'counterbalance in cruise control' to maintain lumen diameter within a designated range. Herein we review recent studies that point to novel arenas for future PC research with therapeutic potential for ADPKD.
Identifiants
pubmed: 36805211
pii: S1471-4914(23)00029-1
doi: 10.1016/j.molmed.2023.01.005
pii:
doi:
Substances chimiques
TRPP Cation Channels
0
Types de publication
Journal Article
Review
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
268-281Informations de copyright
Copyright © 2023 Elsevier Ltd. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of interests The authors have no interests to declare.