Ca2 homeostasis defects and hereditary hearing loss. (Q37893568)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21698697 AND SRC:MED&resulttype=core&format=json

5 February 2020

review article

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title

Ca2 homeostasis defects and hereditary hearing loss (English)

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21698697 AND SRC:MED&resulttype=core&format=json

5 February 2020

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1

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21698697 AND SRC:MED&resulttype=core&format=json

5 February 2020

author name string

Fabio Mammano

series ordinal

1

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24 August 2017

publication date

1 May 2011

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21698697 AND SRC:MED&resulttype=core&format=json

5 February 2020

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21698697 AND SRC:MED&resulttype=core&format=json

5 February 2020

volume

37

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21698697 AND SRC:MED&resulttype=core&format=json

5 February 2020

issue

3

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21698697 AND SRC:MED&resulttype=core&format=json

5 February 2020

page(s)

182-188

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21698697 AND SRC:MED&resulttype=core&format=json

Very low calcium content of cochlear endolymph, an extracellular fluid

5 February 2020

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Coordinated control of connexin 26 and connexin 30 at the regulatory and functional level in the inner ear

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Cochlear gap junctions coassembled from Cx26 and 30 show faster intercellular Ca2 signaling than homomeric counterparts.

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Human connexin26 and connexin30 form functional heteromeric and heterotypic channels

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Function and expression pattern of nonsyndromic deafness genes

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Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell death

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Transgenic expression of a dominant-negative connexin26 causes degeneration of the organ of Corti and non-syndromic deafness

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Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness

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Connexin30 deficiency causes instrastrial fluid-blood barrier disruption within the cochlear stria vascularis

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Novel Mutations in the Connexin 26 Gene (GJB2) That Cause Autosomal Recessive (DFNB1) Hearing Loss

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Loss-of-function and residual channel activity of connexin26 mutations associated with non-syndromic deafness

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Impaired permeability to Ins(1,4,5)P3 in a mutant connexin underlies recessive hereditary deafness

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Gap junctions: getting the message through

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Connexin channel permeability to cytoplasmic molecules

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New roles for astrocytes: Gap junction hemichannels have something to communicate

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Beyond the gap: functions of unpaired connexon channels

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ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2 signals across the inner ear

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Vesicular storage of adenosine triphosphate in the guinea-pig cochlear lateral wall and concentrations of ATP in the endolymph during sound exposure and hypoxia

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A mechanism for sensing noise damage in the inner ear.

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ATP-mediated cell-cell signaling in the organ of Corti: the role of connexin channels

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Mapping and characterization of the basal promoter of the human connexin26 gene

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The TLR3 ligand polyI: C downregulates connexin 43 expression and function in astrocytes by a mechanism involving the NF-kappaB and PI3 kinase pathways

21 January 2018

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The human deafness-associated connexin 30 T5M mutation causes mild hearing loss and reduces biochemical coupling among cochlear non-sensory cells in knock-in mice

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The origin of spontaneous activity in the developing auditory system.

21 January 2018

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Developmental regulation of spontaneous activity in the Mammalian cochlea

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Calcium action potentials in hair cells pattern auditory neuron activity before hearing onset

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Cross-links between stereocilia in the guinea pig organ of Corti, and their possible relation to sensory transduction

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Localization of inner hair cell mechanotransducer channels using high-speed calcium imaging.

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Calcium imaging of single stereocilia in hair cells: localization of transduction channels at both ends of tip links

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The concentrations of calcium buffering proteins in mammalian cochlear hair cells

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mdfw:A Deafness Susceptibility Locus That Interacts with Deaf Waddler (dfw)

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Balance and hearing deficits in mice with a null mutation in the gene encoding plasma membrane Ca2 -ATPase isoform 2

21 January 2018

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Mutations in a plasma membrane Ca2 -ATPase gene cause deafness in deafwaddler mice

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A point mutation in a plasma membrane Ca(2 )-ATPase gene causes deafness in Wriggle Mouse Sagami

21 January 2018

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Haplo-insufficiency revealed in deafwaddler mice when tested for hearing loss and ataxia

21 January 2018

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A de novo deafwaddler mutation of Pmca2 arising in ES cells and hitchhiking with a targeted modification of the Pparg gene

21 January 2018

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The novel mouse mutation Oblivion inactivates the PMCA2 pump and causes progressive hearing loss

21 January 2018

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Modification of human hearing loss by plasma-membrane calcium pump PMCA2

21 January 2018

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A functional study of plasma-membrane calcium-pump isoform 2 mutants causing digenic deafness

21 January 2018

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The novel PMCA2 pump mutation Tommy impairs cytosolic calcium clearance in hair cells and links to deafness in mice.

21 January 2018

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Calcium pump kinetics determined in single erythrocyte ghosts by microphotolysis and confocal imaging

21 January 2018

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Low endolymph calcium concentrations in deafwaddler2J mice suggest that PMCA2 contributes to endolymph calcium maintenance

21 January 2018

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Structural determinants of cadherin-23 function in hearing and deafness

21 January 2018

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Cadherin 23 and protocadherin 15 interact to form tip-link filaments in sensory hair cells

21 January 2018

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Linking genes underlying deafness to hair-bundle development and function

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A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growth

21 January 2018

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Tip-link integrity and mechanical transduction in vertebrate hair cells

21 January 2018

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The micromachinery of mechanotransduction in hair cells

21 January 2018

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Steady-state adaptation of mechanotransduction modulates the resting potential of auditory hair cells, providing an assay for endolymph [Ca2 ].

21 January 2018

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Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss

21 January 2018

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Gap Junction Systems in the Rat Vestibular Labyrinth: Immunohistochemical and Ultrastructural Analysis

21 January 2018

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Connexins Responsible for Hereditary Deafness — The Tale Unfolds

21 January 2018

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