Search Results - "MICHALSKI, Nicolas"
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Mapping the Fine-Scale Organization and Plasticity of the Brain Vasculature
Published in Cell (20-02-2020)“…The cerebral vasculature is a dense network of arteries, capillaries, and veins. Quantifying variations of the vascular organization across individuals, brain…”
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Stiffness and tension gradients of the hair cell's tip-link complex in the mammalian cochlea
Published in eLife (01-04-2019)“…Sound analysis by the cochlea relies on frequency tuning of mechanosensory hair cells along a tonotopic axis. To clarify the underlying biophysical mechanism,…”
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3
BMP signaling specifies the development of a large and fast CNS synapse
Published in Nature neuroscience (01-07-2013)“…The calyx of Held synapse in the auditory brainstem is an unusually large and fast synapse. Using genome-wide screening and conditional deletion in mice, Xiao…”
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Control of exocytosis by synaptotagmins and otoferlin in auditory hair cells
Published in The Journal of neuroscience (06-10-2010)“…In pre-hearing mice, vesicle exocytosis at cochlear inner hair cell (IHC) ribbon synapses is triggered by spontaneous Ca(2+) spikes. At the onset of hearing,…”
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Defect in the gene encoding the EAR/EPTP domain-containing protein TSPEAR causes DFNB98 profound deafness
Published in Human molecular genetics (01-09-2012)“…We report a consanguineous Iranian family affected by congenital profound sensorineural deafness segregating in an autosomal recessive mode. Auditory tests…”
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Otoferlin acts as a Ca2+ sensor for vesicle fusion and vesicle pool replenishment at auditory hair cell ribbon synapses
Published in eLife (07-11-2017)“…Hearing relies on rapid, temporally precise, and sustained neurotransmitter release at the ribbon synapses of sensory cells, the inner hair cells (IHCs). This…”
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Auditory cortex interneuron development requires cadherins operating hair-cell mechanoelectrical transduction
Published in Proceedings of the National Academy of Sciences - PNAS (25-07-2017)“…Many genetic forms of congenital deafness affect the sound reception antenna of cochlear sensory cells, the hair bundle. The resulting sensory deprivation…”
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8
An organotypic slice culture to study the formation of calyx of Held synapses in-vitro
Published in PloS one (18-04-2017)“…The calyx of Held, a large axo-somatic relay synapse containing hundreds of presynaptic active zones, is possibly the largest nerve terminal in the mammalian…”
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Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane
Published in Journal of comparative neurology (1911) (01-02-2011)“…Stereocilin is defective in a recessive form of deafness, DFNB16. We studied the distribution of stereocilin in the developing and mature mouse inner ear and…”
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10
Usher type 1G protein sans is a critical component of the tip-link complex, a structure controlling actin polymerization in stereocilia
Published in Proceedings of the National Academy of Sciences - PNAS (05-04-2011)“…The mechanotransducer channels of auditory hair cells are gated by tip-links, oblique filaments that interconnect the stereocilia of the hair bundle. Tip-links…”
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Spontaneous Mouse Behavior in Presence of Dissonance and Acoustic Roughness
Published in Frontiers in behavioral neuroscience (08-10-2020)“…According to a novel hypothesis (Arnal et al., 2015, Current Biology 25:2051-2056), auditory roughness, or temporal envelope modulations between 30 and 150 Hz,…”
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12
Detecting Central Auditory Processing Disorders in Awake Mice
Published in Brain sciences (31-10-2023)“…Mice are increasingly used as models of human-acquired neurological or neurodevelopmental conditions, such as autism, schizophrenia, and Alzheimer’s disease…”
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The SNARE protein SNAP-25 is required for normal exocytosis at auditory hair cell ribbon synapses
Published in iScience (22-12-2022)“…Hearing depends on fast and sustained calcium-dependent synaptic vesicle fusion at the ribbon synapses of cochlear inner hair cells (IHCs). The implication of…”
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Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly
Published in Human molecular genetics (01-02-2005)“…Defects in myosin XVa and the PDZ domain-containing protein, whirlin, underlie deafness in humans and mice. Hair bundles of mutant mice defective for either…”
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Central auditory deficits associated with genetic forms of peripheral deafness
Published in Human genetics (01-04-2022)“…Since the 1990s, the study of inherited hearing disorders, mostly those detected at birth, in the prelingual period or in young adults, has led to the…”
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Genes Involved in the Development and Physiology of Both the Peripheral and Central Auditory Systems
Published in Annual review of neuroscience (08-07-2019)“…The genetic approach, based on the study of inherited forms of deafness, has proven to be particularly effective for deciphering the molecular mechanisms…”
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Genetics of auditory mechano-electrical transduction
Published in Pflügers Archiv (01-01-2015)“…The hair bundles of cochlear hair cells play a central role in the auditory mechano-electrical transduction (MET) process. The identification of MET components…”
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Characterizing subcutaneous cortical auditory evoked potentials in mice
Published in Hearing research (01-09-2022)“…Auditory Brainstem Responses (ABRs) are a reliably robust measure of auditory thresholds in the mammalian hearing system and can be used to determine deficits…”
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Single-cell transcriptomic profiling of the mouse cochlea: An atlas for targeted therapies
Published in Proceedings of the National Academy of Sciences - PNAS (27-06-2023)“…Functional molecular characterization of the cochlea has mainly been driven by the deciphering of the genetic architecture of sensorineural deafness. As a…”
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Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis
Published in Proceedings of the National Academy of Sciences - PNAS (08-12-2020)“…Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors…”
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