Piezo1
Family:
Piezo channels; piezo-type mechanosensitive ion channel.
Members:
Piezo1 and Piezo2 in vertebrates, encoded by PIEZO1 and PIEZO2 genes, respectively.
Topology:
Piezo 1 is a trimeric mechanosensitive ion channel.Piezos are very large proteins with numerous (>14) predicted transmembrane (TM) domains per subunit.
Regulation and Function:
Piezo1 opens in response to mechanical stimuli such as shear stress and membrane stretch, allowing positively charged ions, including calcium, to flow into the cell.
Piezo1: Background information
Overview:
Piezo proteins are the pore-forming subunits of trimeric mechanosensitive ion channels that open in response to mechanical stimuli such as shear stress and membrane stretch, allowing positively charged ions, including calcium, to flow into the cell.
Data Sheet:
Gene:
PIEZO1
Human Protein:
UniProt Q92508
Tissue:
Expressed in tissues of hollow organs such as stomach, lungs, bladder, intestines, and endothelial cells lining the lumen of blood vessels. Also expressed red blood cells.
Function and pathology:
Gain-of-function (GOF) mutations in human Piezo1 cause hereditary xerocytosis (also known as dehydrated stomatocytosis), a familial anemia. Loss-of-function (LOF) mutations cause generalized lymphatic dysplasia characterized by varying degrees of anemia. Both channelopathies suggest a central role that Piezo1 plays in erythrocyte volume control.
Pathology:
Hereditary xerocytosis
Selective activators:
Yoda1; jedi1 (mouse); jedi1 (mouse)
Inhibitors:
Dooku1; ruthenium red; gadolinium
Assays:
Patch Clamp: whole cell, single channel, bilayers, mechanosensitive activation
Application Notes
Data and Applications
Webinars
Podcast
Introducing Professor Lars Kaestner (Saarland University) - Red Blood Cells
In this edition of the podcast - we speak to Prof. Lars Kaestner and his work with Red Blood Cells; specifically looking at advances in diagnostic tools for Neuroacanthocytosis.