SELECTED PUBLICATIONS

Immunoflorescence detection of TMC1 in Loxhd1-deficient hair bundles.

LOXHD1 is indispensable for maintaining TMC1 auditory mechanosensitive channels at the site of force transmission.

Wang P, Miller KK, He E, Dhawan SS, Cunningham CL, Grillet N.

Nat Commun. 2024 Sep 10;15(1):7865.

PMID: 39256406 Journal Featured

Scanning electron microscope images of gold beads labeling MYO15A.

SUB-immunogold-SEM reveals nanoscale distribution of submembranous epitopes.

Miller KK, Wang P, Grillet N.

Nat Commun. 2024 Sep 10;15(1):7864.

PMID: 39256352 Journal

Scanning electron microscope images of cochlear hair cells of a vestibular Schwannoma patient.

Single-cell transcriptomic atlas reveals increased regeneration in diseased human inner ear balance organs.

Wang T, Ling AH, Billings SE, Hosseini DK, Vaisbuch Y, Kim GS, Atkinson PJ, Sayyid ZN, Aaron KA, Wagh D, Pham N, Scheibinger M, Zhou R, Ishiyama A, Moore LS, Maria PS, Blevins NH, Jackler RK, Alyono JC, Kveton J, Navaratnam D, Heller S, Lopez IA, Grillet N., Jan TA, Cheng AG.

Nat Commun. 2024 Jun 6;15(1):4833.

PMID: 38844821 Journal

Diagram illustrating the genetic pathway involved in Ewing sarcoma, highlighting the roles of big data, EWS Signature ESS32, LOXHD1, and various genes, alongside effects on cell proliferation, cytoskeleton, and tumor progression.

Oncofusion-driven de novo enhancer assembly promotes malignancy in Ewing sarcoma via aberrant expression of the stereociliary protein LOXHD1.

Deng Q, Natesan R, Cidre-Aranaz F, Arif S, Liu Y, Rasool RU, Wang P, Mitchell-Velasquez E, Das CK, Vinca E, Cramer Z, Grohar PJ, Chou M, Kumar-Sinha C, Weber K, Eisinger-Mathason TSK, Grillet N., Grünewald TGP, Asangani IA.

Cell Rep. 2022 Jun 14;39(11):110971.

PMID: 35705030 Journal

Graph comparing the height and percentage of live cells in samples prepared with Mild fixation using Phalloidin-488, Live Di-4, and SEM prep, showing mean percentages and variability.

Dimensions of a Living Cochlear Hair Bundle.

Miller KK, Atkinson P, Mendoza KR, Ó Maoiléidigh D*, Grillet N*.

Front Cell Dev Biol. 2021;9:742529.

PMID: 34900993 Journal

Inner hair cells mechanotransduction currents at P7 and P11 stages in controled and Loxhd1-mutants.

Loxhd1 Mutations Cause Mechanotransduction Defects in Cochlear Hair Cells.

Trouillet A, Miller KK, George SS, Wang P, Ali NE, Ricci A, Grillet N.

J Neurosci. 2021 Apr 14;41(15):3331-3343.

PMID: 33707295 Journal Cover article

Microscopic images showing Piezo2 expression in the brain and spinal cord, with arrows indicating specific areas outer hair cells

Mechanosensory hair cells express two molecularly distinct mechanotransduction channels.

Wu Z*, Grillet N* Zhao B, Cunningham C, Harkins-Perry S, Coste B, Ranade S, Zebarjadi N, Beurg M, Fettiplace R, Patapoutian A, Mueller U.

Nat Neurosci. 2017 Jan;20(1):24-33.

PMID: 27893727 Journal

Diagram showing gene sequencing with chromatograms for wild type and audio-1 gene, highlighting a specific mutation in Nptn gene.

Neuroplastin Isoform Np55 Is Expressed in the Stereocilia of Outer Hair Cells and Required for Normal Outer Hair Cell Function.

Zeng WZ*, Grillet N*, Dewey JB, Trouillet A, Krey JF, Barr-Gillespie PG, Oghalai JS, Müller U.

J Neurosci. 2016 Aug 31;36(35):9201-16.

PMID: 27581460 Journal

Diagram of a cell membrane showing protein complexes, receptors labeled PCDH15 CD1/CD3 and CD2, with TMIE and LHFPL5,/TMHS.

TMIE is an essential component of the mechanotransduction machinery of cochlear hair cells.

Zhao B, Wu Z, Grillet N., Yan L, Xiong W, Harkins-Perry S, Müller U.

Neuron. 2014 Dec 3;84(5):954-67.

PMID: 25467981 Journal

Scanning electron microscope images of cochlear hair bundles, with stereocilia colored by rows.

TMHS is an integral component of the mechanotransduction machinery of cochlear hair cells.

Xiong W, Grillet N, Elledge HM, Wagner TF, Zhao B, Johnson KR, Kazmierczak P, Müller U.

Cell. 2012 Dec 7;151(6):1283-95.

PMID: 23217710   Journal

A scientific figure with two panels showing immunohistochemistry results in mouse tissue samples. Panel A compares wild type (wt) mice and control, with graphs indicating different levels of 5P probe, CD1, CD2, and CD3 markers. Panel B shows images of tissue from mice with deleted CD1, CD2, and CD3 genes, with arrows pointing to specific regions. Each marker is color-coded and indicates levels of expression.

Regulation of PCDH15 function in mechanosensory hair cells by alternative splicing of the cytoplasmic domain.

Webb SW, Grillet N, Andrade LR, Xiong W, Swarthout L, Della Santina CC, Kachar B, Müller U.

Development. 2011 Apr;138(8):1607-17.

PMID: 21427143   Journal

Pedigree chart showing family genetic inheritance patterns, with black symbols indicating ARNSHL genetic disorder and hLOXHD1 genotypes.

Mutations in LOXHD1, an evolutionarily conserved stereociliary protein, disrupt hair cell function in mice and cause progressive hearing loss in humans.

Grillet N*, Schwander M*, Hildebrand MS, Sczaniecka A, Kolatkar A, Velasco J, Webster JA, Kahrizi K, Najmabadi H, Kimberling WJ, Stephan D, Bahlo M, Wiltshire T, Tarantino LM, Kuhn P, Smith RJ, Müller U.

Am J Hum Genet. 2009 Sep;85(3):328-37.

PMID: 19732867   Journal

Transmission Electron microscope images showing the immunogold-localization of Harmonin

Harmonin mutations cause mechanotransduction defects in cochlear hair cells.

Grillet N*, Xiong W*, Reynolds A*, Kazmierczak P, Sato T, Lillo C, Dumont RA, Hintermann E, Sczaniecka A, Schwander M, Williams D, Kachar B, Gillespie PG, Müller U.

Neuron. 2009 May 14;62(3):375-87.

PMID: 19447093   Journal Cover Article Previewed Article

PROTOCOLS

Flowchart depicting a multi-step process for preparing mouse inner ear samples for imaging. Step 1 involves mouse inner ear extraction, fixation, dissection, and strong fixation, taking 3 hours. Step 2 is sample dehydration and critical point drying, taking 2.5 hours. Step 3 is dissection, mounting, and metal coating, taking 24 hours. Step 4 is SEM microscopy imaging, taking 5 hours.

High-resolution imaging of the mouse-hair-cell hair bundle by scanning electron microscopy.

Grillet N.

STAR Protoc. 2022 Mar 18;3(1):101213.

PMID: 35257116 Journal

Flowchart illustrating the process of preparing mouse inner ear tissues for imaging, including inner ear extraction, cochlea dissection, immunostaining, and high-resolution fluorescence imaging, with times for each step.

High-resolution immunofluorescence imaging of mouse cochlear hair bundles.

Miller KK, Wang P, Grillet N.

STAR Protoc. 2022 Jun 17;3(2):101431.

PMID: 35669049 Journal

REVIEWS

quote about the physiology of hearing loss and the function of the auditory system.

The genetics of progressive hearing loss: a link between hearing impairment and dysfunction of mechanosensory hair cells.

Müller U, Grillet N.

Future Neurol. 2010 Jan 1;5(1):9-12.

PMID: 24436636   Journal

Review in Science Signaling on Auditory Mechanotransduction.

The mechanotransduction machinery of hair cells.

Grillet N, Kazmierczak P, Xiong W, Schwander M, Reynolds A, Sakaguchi H, Tokita J, Kachar B, Müller U.

Sci Signal. 2009 Aug 25;2(85):pt5.

PMID: 19706872   Journal