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https://www.readbyqxmd.com/read/28728825/genetic-diseases-of-piezo1-and-piezo2-dysfunction
#1
S L Alper
Mutations in the genes encoding the mechanosensitive cation channels PIEZO1 and PIEZO2 are responsible for multiple hereditary human diseases. Loss-of-function mutations in the human PIEZO1 gene cause autosomal recessive congenital lymphatic dysplasia. Gain-of-function mutations in the human PIEZO1 gene cause the autosomal dominant hemolytic anemia, hereditary xerocytosis (also known as dehydrated stomatocytosis). Loss-of-function mutations in the human PIEZO2 gene cause an autosomal recessive syndrome of muscular atrophy with perinatal respiratory distress, arthrogryposis, and scoliosis...
2017: Current Topics in Membranes
https://www.readbyqxmd.com/read/28728820/role-of-piezo-channels-in-joint-health-and-injury
#2
W Lee, F Guilak, W Liedtke
Cartilage is an intrinsically mechanically sensitive tissue composed of chondrocytes as the only cell type. Chondrocyte mechanotransduction is not well understood, but recently we identified critical components of the mechanotransduction machinery demonstrating how mechanical stimulation of these cells can be converted into cellular calcium signals. Physiologic mechanical cues induce anabolic responses of (post-mitotic) chondrocytes via transient receptor potential vanilloid 4 ion channels, whereas injurious mechanical stress is transduced by Piezo1 jointly with Piezo2 ion channels...
2017: Current Topics in Membranes
https://www.readbyqxmd.com/read/28728819/regulation-of-piezo-channels-by-cellular-signaling-pathways
#3
I Borbiro, T Rohacs
The recently identified mechanically activated Piezo1 and Piezo2 channels play major roles in various aspects of mechanosensation in mammals, and their mutations are associated with human diseases. Recent reports show that activation of cell surface receptors coupled to heterotrimeric Gq proteins increase the sensitivity of Piezo2 channels to mechanical stimuli. Activation of the cyclic adenosine monophosphate pathway was also shown to potentiate Piezo2 channel activity. This phenomenon may play a role in mechanical allodynia or hyperalgesia during inflammation...
2017: Current Topics in Membranes
https://www.readbyqxmd.com/read/28728818/mechanosensitive-piezo-channels-in-the-gastrointestinal-tract
#4
C Alcaino, G Farrugia, A Beyder
Sensation of mechanical forces is critical for normal function of the gastrointestinal (GI) tract and abnormalities in mechanosensation are linked to GI pathologies. In the GI tract there are several mechanosensitive cell types-epithelial enterochromaffin cells, intrinsic and extrinsic enteric neurons, smooth muscle cells and interstitial cells of Cajal. These cells use mechanosensitive ion channels that respond to mechanical forces by altering transmembrane ionic currents in a process called mechanoelectrical coupling...
2017: Current Topics in Membranes
https://www.readbyqxmd.com/read/28728817/piezo2-in-cutaneous-and-proprioceptive-mechanotransduction-in-vertebrates
#5
E O Anderson, E R Schneider, S N Bagriantsev
Mechanosensitivity is a fundamental physiological capacity, which pertains to all life forms. Progress has been made with regard to understanding mechanosensitivity in bacteria, flies, and worms. In vertebrates, however, the molecular identity of mechanotransducers in somatic and neuronal cells has only started to appear. The Piezo family of mechanogated ion channels marks a pivotal milestone in understanding mechanosensitivity. Piezo1 and Piezo2 have now been shown to participate in a number of processes, ranging from arterial modeling to sensing muscle stretch...
2017: Current Topics in Membranes
https://www.readbyqxmd.com/read/28728816/in-touch-with-the-mechanosensitive-piezo-channels-structure-ion-permeation-and-mechanotransduction
#6
J Geng, Q Zhao, T Zhang, B Xiao
Mechanotransduction, the conversion of mechanical forces into biological signals, plays critical roles in various physiological and pathophysiological processes in mammals, such as conscious sensing of touch, pain, and sound, as well as unconscious sensing of blood flow-associated shear stress, urine flow, and bladder distention. Among the various molecules involved in mechanotransduction, mechanosensitive (MS) cation channels have long been postulated to represent one critical class of mechanotransducers that directly and rapidly converts mechanical force into electrochemical signals...
2017: Current Topics in Membranes
https://www.readbyqxmd.com/read/28728814/a-tour-de-force-the-discovery-properties-and-function-of-piezo-channels
#7
P A Gottlieb
Mechanical transducers appear throughout cell biology and are used to convert mechanical stress into chemical or electrical signals that allow the cell to respond to environmental changes. In the past six years, a eukaryotic mechanical channel family with two members, Piezo1 and Piezo2, has been identified. Piezo1 was shown to be a cation-selective channel that does not require ancillary proteins for activity. Mouse Piezo1 is large, with over 2500 amino acids, and is not homologous to other ion channels. Both piezo channels have rapid voltage-dependent inactivation with a reversal potential near 0mV...
2017: Current Topics in Membranes
https://www.readbyqxmd.com/read/28636944/transduction-of-repetitive-mechanical-stimuli-by-piezo1-and-piezo2-ion-channels
#8
Amanda H Lewis, Alisa F Cui, Malcolm F McDonald, Jörg Grandl
Several cell types experience repetitive mechanical stimuli, including vein endothelial cells during pulsating blood flow, inner ear hair cells upon sound exposure, and skin cells and their innervating dorsal root ganglion (DRG) neurons when sweeping across a textured surface or touching a vibrating object. While mechanosensitive Piezo ion channels have been clearly implicated in sensing static touch, their roles in transducing repetitive stimulations are less clear. Here, we perform electrophysiological recordings of heterologously expressed mouse Piezo1 and Piezo2 responding to repetitive mechanical stimulations...
June 20, 2017: Cell Reports
https://www.readbyqxmd.com/read/28545921/a-mechanically-activated-ion-channel-is-functionally-expressed-in-the-mrgprb4-positive-sensory-neurons-which-detect-stroking-of-hairy-skin-in-mice
#9
Soichiro Yamaguchi, Ken-Ichi Otsuguro
Mas-related G-protein coupled receptor B4 (MrgprB4) has been reported to be expressed in the dorsal root ganglion (DRG) neurons which detect stroking of hairy skin of mice. However, the mechanisms by which the MrgprB4 positive (+) neurons respond to adequate stimulus remain unsolved as it was also reported that electrophysiological analysis of cultured MrgprB4+ neurons did not reveal responses to mechanical stimuli. Contrary to the observation, however, in this study we show that the MrgprB4+ neurons functionally express a mechanically activated channel using DRG neurons dissociated from genetically-modified mice whose MrgprB4+ neurons express a red fluorescent protein...
July 13, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28527849/role-of-piezo-channels-in-ultrasound-stimulated-dental-stem-cells
#10
Qianhua Gao, Paul R Cooper, A Damien Walmsley, Ben A Scheven
INTRODUCTION: Piezo1 and Piezo2 are mechanosensitive membrane ion channels. We hypothesized that Piezo proteins may play a role in transducing ultrasound-associated mechanical signals and activate downstream mitogen-activated protein kinase (MAPK) signaling processes in dental cells. In this study, the expression and role of Piezo channels were investigated in dental pulp stem cells (DPSCs) and periodontal ligament stem cells (PDLSCs) after treatment with low-intensity pulsed ultrasound (LIPUS)...
May 17, 2017: Journal of Endodontics
https://www.readbyqxmd.com/read/28388364/piezo2-expression-in-mechanosensitive-dental-primary-afferent-neurons
#11
J Won, H Vang, P R Lee, Y H Kim, H W Kim, Y Kang, S B Oh
Mechanosensitive ion channels have been suggested to be expressed in dental primary afferent (DPA) neurons to transduce the movement of dentinal fluid since the proposal of hydrodynamic theory. Piezo2, a mechanosensitive, rapidly inactivating (RI) ion channel, has been recently identified in dorsal root ganglion (DRG) neurons to mediate tactile transduction. Here, we examined the expression of Piezo2 in DPA neurons by in situ hybridization, single-cell reverse transcriptase polymerase chain reaction, and whole-cell patch-clamp recordings...
April 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/28295390/mechanically-sensitive-a%C3%AE-nociceptors-that-innervate-bone-marrow-respond-to-changes-in-intra-osseous-pressure
#12
Sara Nencini, Jason Ivanusic
KEY POINTS: Sensory neurons that innervate the bone marrow provide the CNS with information about pain associated with bone disease and pathology, but little is known of their function. Here we use a novel in vivo bone-nerve electrophysiological preparation to study how they respond to noxious mechanical stimulation delivered by increasing intra-osseous pressure. We provide evidence that sensory neurons that innervate the bone marrow respond to high threshold noxious mechanical stimulation, have response properties consistent with a role in nociception, provide information about different features of an intra-osseous pressure stimulus and express the Piezo2 mechano-transducer molecule...
July 1, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28283553/genome-wide-association-study-of-sepsis-in-extremely-premature-infants
#13
Lakshmi Srinivasan, Grier Page, Haresh Kirpalani, Jeffrey C Murray, Abhik Das, Rosemary D Higgins, Waldemar A Carlo, Edward F Bell, Ronald N Goldberg, Kurt Schibler, Beena G Sood, David K Stevenson, Barbara J Stoll, Krisa P Van Meurs, Karen J Johnson, Joshua Levy, Scott A McDonald, Kristin M Zaterka-Baxter, Kathleen A Kennedy, Pablo J Sánchez, Shahnaz Duara, Michele C Walsh, Seetha Shankaran, James L Wynn, C Michael Cotten
OBJECTIVE: To identify genetic variants associated with sepsis (early-onset and late-onset) using a genome-wide association (GWA) analysis in a cohort of extremely premature infants. STUDY DESIGN: Previously generated GWA data from the Neonatal Research Network's anonymised genomic database biorepository of extremely premature infants were used for this study. Sepsis was defined as culture-positive early-onset or late-onset sepsis or culture-proven meningitis. Genomic and whole-genome-amplified DNA was genotyped for 1...
March 10, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/28185323/an-ultrasensitive-visco-poroelastic-artificial-mechanotransducer-skin-inspired-by-piezo2-protein-in-mammalian-merkel-cells
#14
Ming Liang Jin, Sangsik Park, Younghoon Lee, Ji Hye Lee, Junho Chung, Joo Sung Kim, Jong-Seon Kim, So Young Kim, Eunsong Jee, Dae Woo Kim, Jae Woo Chung, Seung Geol Lee, Dukhyun Choi, Hee-Tae Jung, Do Hwan Kim
An artificial ionic mechanotransducer skin with an unprecedented sensitivity over a wide spectrum of pressure by fabricating visco-poroelastic nanochannels and microstructured features, directly mimicking the physiological tactile sensing mechanism of Piezo2 protein is demonstrated. This capability enables voice identification, health monitoring, daily pressure measurements, and even measurements of a heavy weight beyond capabilities of human skin.
April 2017: Advanced Materials
https://www.readbyqxmd.com/read/28044050/piezo2-a-candidate-biomarker-for-visceral-hypersensitivity-in-irritable-bowel-syndrome
#15
Tao Bai, Ying Li, Jing Xia, Yudong Jiang, Lei Zhang, Huan Wang, Wei Qian, Jun Song, Xiaohua Hou
Background/Aims: Currently, there exists no biomarker for visceral hypersensitivity in irritable bowel syndrome (IBS). Piezo proteins have been proven to play an important role in the mechanical stimulation to induce visceral pain in other tissues and may also be a biomarker candidate. The aim of this study was to test the expressions of Piezo1 and Piezo2 proteins in the intestinal epithelial cells from different intestinal segments and to explore the correlation between Piezo proteins expression and visceral pain threshold...
July 30, 2017: Journal of Neurogastroenterology and Motility
https://www.readbyqxmd.com/read/28039570/different-transcriptional-profiling-between-senescent-and-non-senescent-human-coronary-artery-endothelial-cells-hcaecs-by-omeprazole-and-lansoprazole-treatment
#16
Laura Costarelli, Robertina Giacconi, Marco Malavolta, Andrea Basso, Francesco Piacenza, Mauro Provinciali, Marcello G Maggio, Andrea Corsonello, Fabrizia Lattanzio
Recent evidence suggests that high dose and/or long term use of proton pump inhibitors (PPIs) may increase the risk of adverse cardiovascular events in older patients, but mechanisms underlying these detrimental effects are not known. Taking into account that the senescent endothelial cells have been implicated in the genesis or promotion of age-related cardiovascular disease, we hypothesized an active role of PPIs in senescent cells. The aim of this study is to investigate the changes in gene expression occurring in senescent and non-senescent human coronary artery endothelial cells (HCAECs) following Omeprazole (OPZ) or Lansoprazole (LPZ) treatment...
December 30, 2016: Biogerontology
https://www.readbyqxmd.com/read/28002412/piezo2-senses-airway-stretch-and-mediates-lung-inflation-induced-apnoea
#17
Keiko Nonomura, Seung-Hyun Woo, Rui B Chang, Astrid Gillich, Zhaozhu Qiu, Allain G Francisco, Sanjeev S Ranade, Stephen D Liberles, Ardem Patapoutian
Respiratory dysfunction is a notorious cause of perinatal mortality in infants and sleep apnoea in adults, but the mechanisms of respiratory control are not clearly understood. Mechanical signals transduced by airway-innervating sensory neurons control respiration; however, the physiological significance and molecular mechanisms of these signals remain obscured. Here we show that global and sensory neuron-specific ablation of the mechanically activated ion channel Piezo2 causes respiratory distress and death in newborn mice...
January 12, 2017: Nature
https://www.readbyqxmd.com/read/27974811/recessive-piezo2-stop-mutation-causes-distal-arthrogryposis-with-distal-muscle-weakness-scoliosis-and-proprioception-defects
#18
Goknur Haliloglu, Kerstin Becker, Cagri Temucin, Beril Talim, Nalan Küçükşahin, Matthias Pergande, Susanne Motameny, Peter Nürnberg, Ustun Aydingoz, Haluk Topaloglu, Sebahattin Cirak
The genetic work-up of arthrogryposis is challenging due to the diverse clinical and molecular etiologies. We report a-18(3/12)-year-old boy, from a 2nd degree consanguineous family, who presented at 3(6/12) years with hypotonia, distal laxity, contractures, feeding difficulties at birth. He required surgery for progressive scoliosis at 16 years of age, and walked independently since then with an unstable gait and coordination defects. His latest examination at 18 years of age revealed a proprioceptive defect and loss-of-joint position sense in the upper limbs...
April 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/27912047/biallelic-loss-of-proprioception-related-piezo2-causes-muscular-atrophy-with-perinatal-respiratory-distress-arthrogryposis-and-scoliosis
#19
Andrea Delle Vedove, Markus Storbeck, Raoul Heller, Irmgard Hölker, Malavika Hebbar, Anju Shukla, Olafur Magnusson, Sebahattin Cirak, Katta M Girisha, Mary O'Driscoll, Bart Loeys, Brunhilde Wirth
No abstract text is available yet for this article.
December 1, 2016: American Journal of Human Genetics
https://www.readbyqxmd.com/read/27893727/mechanosensory-hair-cells-express-two-molecularly-distinct-mechanotransduction-channels
#20
Zizhen Wu, Nicolas Grillet, Bo Zhao, Christopher Cunningham, Sarah Harkins-Perry, Bertrand Coste, Sanjeev Ranade, Navid Zebarjadi, Maryline Beurg, Robert Fettiplace, Ardem Patapoutian, Ulrich Mueller
Auditory hair cells contain mechanotransduction channels that rapidly open in response to sound-induced vibrations. We report here that auditory hair cells contain two molecularly distinct mechanotransduction channels. One ion channel is activated by sound and is responsible for sensory transduction. This sensory transduction channel is expressed in hair cell stereocilia, and previous studies show that its activity is affected by mutations in the genes encoding the transmembrane proteins TMHS, TMIE, TMC1 and TMC2...
January 2017: Nature Neuroscience
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