User profiles for Jacques Boutet de Monvel

Jacques Boutet de Monvel

Institut Pasteur
Verified email at pasteur.fr
Cited by 1958

How the genetics of deafness illuminates auditory physiology

GP Richardson, JB de Monvel… - Annual review of …, 2011 - annualreviews.org
Although the basic principles underlying the function of the peripheral auditory system have
been known for many years, the molecules required for hearing have hitherto remained …

Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model

…, S Nouaille, J Boutet de Monvel… - Proceedings of the …, 2019 - National Acad Sciences
Autosomal recessive genetic forms (DFNB) account for most cases of profound congenital
deafness. Adeno-associated virus (AAV)-based gene therapy is a promising therapeutic option…

[HTML][HTML] Image restoration for confocal microscopy: improving the limits of deconvolution, with application to the visualization of the mammalian hearing organ

JB De Monvel, S Le Calvez, M Ulfendahl - Biophysical Journal, 2001 - cell.com
Deconvolution algorithms have proven very effective in conventional (wide-field)
fluorescence microscopy. Their application to confocal microscopy is hampered, in biological …

Otoferlin acts as a Ca2+ sensor for vesicle fusion and vesicle pool replenishment at auditory hair cell ribbon synapses

…, JD Goutman, SM Auclair, J Boutet de Monvel… - Elife, 2017 - elifesciences.org
10.7554/eLife.31013.001 Hearing relies on rapid, temporally precise, and sustained
neurotransmitter release at the ribbon synapses of sensory cells, the inner hair cells (IHCs). This …

Local gene therapy durably restores vestibular function in a mouse model of Usher syndrome type 1G

…, A Lelli, O Akil, J Boutet de Monvel… - Proceedings of the …, 2017 - National Acad Sciences
Our understanding of the mechanisms underlying inherited forms of inner ear deficits has
considerably improved during the past 20 y, but we are still far from curative treatments. We …

Organ of Corti potentials and the motion of the basilar membrane

A Fridberger, JB De Monvel, J Zheng, N Hu… - Journal of …, 2004 - Soc Neuroscience
During sound stimulation, receptor potentials are generated within the sensory hair cells of
the cochlea. Prevailing theory states that outer hair cells use the potential-sensitive motor …

Imaging hair cell transduction at the speed of sound: dynamic behavior of mammalian stereocilia

…, M Ulfendahl, J Boutet de Monvel - Proceedings of the …, 2006 - National Acad Sciences
The cochlea contains two types of sensory cells, the inner and outer hair cells. Sound-evoked
deflection of outer hair cell stereocilia leads to fast force production that will enhance …

[HTML][HTML] Image-adaptive deconvolution for three-dimensional deep biological imaging

JB de Monvel, E Scarfone, S Le Calvez, M Ulfendahl - Biophysical journal, 2003 - cell.com
Deconvolution algorithms are widely used in conventional fluorescence microscopy, but
they remain difficult to apply to deep imaging systems such as confocal and two-photon …

Usher syndrome type 1–associated cadherins shape the photoreceptor outer segment

…, A Estivalet, A Aghaie, J Boutet de Monvel… - Journal of Cell …, 2017 - rupress.org
Usher syndrome type 1 (USH1) causes combined hearing and sight defects, but how mutations
in USH1 genes lead to retinal dystrophy in patients remains elusive. The USH1 protein …

Class III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growth

A Lelli, V Michel, J Boutet de Monvel, M Cortese… - Journal of Cell …, 2016 - rupress.org
The precise architecture of hair bundles, the arrays of mechanosensitive microvilli-like stereocilia
crowning the auditory hair cells, is essential to hearing. Myosin IIIa, defective in the late-…