Increased basal activity is a key determinant in the severity of human skeletal dysplasia caused by TRPV4 mutations
about
Structure of the TRPV1 ion channel determined by electron cryo-microscopyStructural and Biochemical Consequences of Disease-Causing Mutations in the Ankyrin Repeat Domain of the Human TRPV4 ChannelFeeling the hidden mechanical forces in lipid bilayer is an original senseGain-of-function mutation in TRPV4 identified in patients with osteonecrosis of the femoral headFollistatin in chondrocytes: the link between TRPV4 channelopathies and skeletal malformations.Patient-derived skeletal dysplasia induced pluripotent stem cells display abnormal chondrogenic marker expression and regulation by BMP2 and TGFβ1TRPV4-associated skeletal dysplasias.Swelling and eicosanoid metabolites differentially gate TRPV4 channels in retinal neurons and gliaL596-W733 bond between the start of the S4-S5 linker and the TRP box stabilizes the closed state of TRPV4 channel.A channelopathy mechanism revealed by direct calmodulin activation of TrpV4.Human skeletal dysplasia caused by a constitutive activated transient receptor potential vanilloid 4 (TRPV4) cation channel mutation.Unraveling the mechanism by which TRPV4 mutations cause skeletal dysplasias.Novel mutations highlight the key role of the ankyrin repeat domain in TRPV4-mediated neuropathy.A TRPV4 channel C-terminal folding recognition domain critical for trafficking and function.A competing hydrophobic tug on L596 to the membrane core unlatches S4-S5 linker elbow from TRP helix and allows TRPV4 channel to open.The force-from-lipid (FFL) principle of mechanosensitivity, at large and in elements.Yeast luminometric and Xenopus oocyte electrophysiological examinations of the molecular mechanosensitivity of TRPV4.When size matters: transient receptor potential vanilloid 4 channel as a volume-sensor rather than an osmo-sensor.Transient receptor potential vanilloid 4 (TRPV4) activation by arachidonic acid requires protein kinase A-mediated phosphorylation.Sensing and regulation of cell volume - we know so much and yet understand so little: TRPV4 as a sensor of volume changes but possibly without a volume-regulatory role?A mutation in TRPV4 results in altered chondrocyte calcium signaling in severe metatropic dysplasiaFetal akinesia in metatropic dysplasia: The combined phenotype of chondrodysplasia and neuropathy?
P2860
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P2860
Increased basal activity is a key determinant in the severity of human skeletal dysplasia caused by TRPV4 mutations
description
2011 nî lūn-bûn
@nan
2011 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի մայիսին հրատարակված գիտական հոդված
@hy
2011年の論文
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2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
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name
Increased basal activity is a ...... asia caused by TRPV4 mutations
@ast
Increased basal activity is a ...... asia caused by TRPV4 mutations
@en
type
label
Increased basal activity is a ...... asia caused by TRPV4 mutations
@ast
Increased basal activity is a ...... asia caused by TRPV4 mutations
@en
prefLabel
Increased basal activity is a ...... asia caused by TRPV4 mutations
@ast
Increased basal activity is a ...... asia caused by TRPV4 mutations
@en
P2093
P2860
P1433
P1476
Increased basal activity is a ...... asia caused by TRPV4 mutations
@en
P2093
Ching Kung
Stephen Loukin
Zhenwei Su
P2860
P304
P356
10.1371/JOURNAL.PONE.0019533
P407
P577
2011-05-05T00:00:00Z