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Publication
Loss of cilia suppresses cyst growth in genetic models of autosomal dominant
polycystic kidney disease.
Authors Ma M, Tian X, Igarashi P, Pazour GJ, Somlo S
Submitted By Submitted Externally on 8/14/2017
Status Published
Journal Nature genetics
Year 2013
Date Published 9/1/2013
Volume : Pages 45 : 1004 - 12
PubMed Reference 23892607
Abstract Kidney cysts occur following inactivation of polycystins in otherwise intact
cilia or following complete removal of cilia by inactivation of intraflagellar
transport-related proteins. We investigated the mechanisms of cyst formation in
these two distinct processes by combining conditional inactivation of
polycystins with concomitant ablation of cilia in developing and adult kidney
and liver. We found that loss of intact cilia suppressed cyst growth following
inactivation of polycystins and that the severity of cystic disease was directly
related to the length of time between the initial loss of the polycystin
proteins and the subsequent involution of cilia. This cilia-dependent cyst
growth was not explained by activation of the MAPK/ERK, mTOR or cAMP pathways
and is likely to be distinct from the mechanism of cyst growth following
complete loss of cilia. These data establish the existence of a new pathway
defined by polycystin-dependent inhibition and cilia-dependent activation that
promotes rapid cyst growth.








Genes
SymbolDescription
Pkd1polycystic kidney disease 1 homolog
Pkd2polycystic kidney disease 2

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