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Polycystin Channel Complexes
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DOI：10.1146/annurev-physiol-031522-084334.png)
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Abstract
En 中文
Polycystin subunits can form hetero- and homotetrameric ion channels in the membranes of various compartments of the cell. Homotetrameric polycystin channels are voltage- and calcium-modulated, whereas heterotetrameric versions are proposed to be ligand- or autoproteolytically regulated. Their importance is underscored by variants associated with autosomal dominant polycystic kidney disease and by vital roles in fertilization and embryonic development. The diversity in polycystin assembly and subcellular distribution allows for a multitude of sensory functions by this class of channels. In this review, we highlight their recent structural and functional characterization, which has provided a molecular blueprint to investigate the conformational changes required for channel opening in response to unique stimuli. We consider each polycystin channel type individually, discussing how they contribute to sensory cell biology, as well as their impact on the physiology of various tissues.
Keywords:
LEFT-RIGHT ASYMMETRY
FLUID-CONTACTING NEURONS
EF-HAND DOMAIN
KIDNEY-DISEASE
TRANSIENT RECEPTOR
CRYSTAL-STRUCTURE
PROTEIN PRODUCT
CALCIUM-CHANNEL
SODIUM-CHANNEL
SONIC HEDGEHOG
Journal
IF:
19.1
Papers:
821
Citations:
1.2W
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