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B. Delprat · SciRah

Researcher profile · Semantic Scholar

B. Delprat

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B. Delprat — researcher profile on SciRah

h-index: 19
Works: 44
Citations: 1,715

Most-cited papers

Impairment of SLC17A8 encoding vesicular glutamate transporter-3, VGLUT3, underlies nonsyndromic deafness DFNA25 and inner hair cell dysfunction in null mice.
J. Ruel, S. Emery, R. Nouvian, Tiphaine Bersot +13 more
2008American Journal of Human Genetics278 citations Open access
Semantic Scholar
Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly.
B. Delprat, Vincent Michel, R. Goodyear, Yasuhiro Yamasaki +7 more
2005Human Molecular Genetics195 citations Open access
Semantic Scholar
Usherin, the defective protein in Usher syndrome type IIA, is likely to be a component of interstereocilia ankle links in the inner ear sensory cells.
A. Adato, Gaëlle M. Lefevre, B. Delprat, Vincent Michel +5 more
2005Human Molecular Genetics194 citations
Semantic Scholar
ER-mitochondria cross-talk is regulated by the Ca2+ sensor NCS1 and is impaired in Wolfram syndrome
C. Angebault, J. Fauconnier, S. Patergnani, J. Rieusset +15 more
2018Science Signaling136 citations Open access
Semantic Scholar
Wolfram syndrome: MAMs’ connection?
B. Delprat, T. Maurice, C. Delettre
2018Cell Death and Disease83 citations Open access
Semantic Scholar
Tmprss3, a Transmembrane Serine Protease Deficient in Human DFNB8/10 Deafness, Is Critical for Cochlear Hair Cell Survival at the Onset of Hearing*
Lydie Fasquelle, H. Scott, M. Lenoir, Jing Wang +11 more
2011Journal of Biological Chemistry79 citations Open access
Semantic Scholar
FXYD6 Is a Novel Regulator of Na,K-ATPase Expressed in the Inner Ear*
B. Delprat, Danièle Schaer, Sophie Roy, Jing Wang +2 more
2007Journal of Biological Chemistry71 citations Open access
Semantic Scholar
Deafness and Cochlear Fibrocyte Alterations in Mice Deficient for the Inner Ear Protein Otospiralin
B. Delprat, J. Ruel, M. Guitton, G. Hamard +5 more
2005Molecular and Cellular Biology70 citations Open access
Semantic Scholar
Activation of the sigma-1 receptor chaperone alleviates symptoms of Wolfram syndrome in preclinical models
Lucie Crouzier, A. Danese, Yuko Yasui, Elodie M. Richard +12 more
2022Science Translational Medicine69 citations Open access
Semantic Scholar
At the Crossing of ER Stress and MAMs: A Key Role of Sigma-1 Receptor?
B. Delprat, Lucie Crouzier, Tsung-Ping Su, T. Maurice
2020Advances in Experimental Medicine and Biology66 citations
Semantic Scholar
Use of Zebrafish Models to Boost Research in Rare Genetic Diseases
Lucie Crouzier, Elodie M. Richard, J. Sourbron, L. Lagae +2 more
2021International Journal of Molecular Sciences51 citations Open access
Semantic Scholar
Downregulation of Otospiralin, a Novel Inner Ear Protein, Causes Hair Cell Degeneration and Deafness
B. Delprat, A. Boulanger, Jing Wang, V. Beaudoin +7 more
2002Journal of Neuroscience44 citations Open access
Semantic Scholar
A novel heterozygous OPA3 mutation located in the mitochondrial target sequence results in altered steady-state levels and fragmented mitochondrial network
Tanja Grau, L. Burbulla, Gertraud Engl, C. Delettre +8 more
2013Journal of Medical Genetics41 citations Open access
Semantic Scholar
Tmprss3 loss of function impairs cochlear inner hair cell Kcnma1 channel membrane expression.
L. Molina, Lydie Fasquelle, R. Nouvian, N. Salvetat +5 more
2013Human Molecular Genetics33 citations
Semantic Scholar
Sigma-1 (σ1) receptor activity is necessary for physiological brain plasticity in mice.
Lucie Crouzier, S. Couly, Chloé Roques, Coralie Peter +5 more
2020European Neuropsychopharmacology30 citations Open access
Semantic Scholar
Sigma-1 receptor agonist PRE-084 confers protection against TAR DNA-binding protein-43 toxicity through NRF2 signalling
C. Lasbleiz, Amandine Peyrel, P. Tarot, J. Sarniguet +6 more
2022Redox Biology29 citations Open access
Semantic Scholar
Remodeling of the Inner Hair Cell Microtubule Meshwork in a Mouse Model of Auditory Neuropathy AUNA1
C. Surel, Marie Guillet, M. Lenoir, J. Bourien +6 more
2016eNeuro24 citations Open access
Semantic Scholar
The Wolfram-like variant WFS1E864K destabilizes MAM and compromises autophagy and mitophagy in human and mice
S. Patergnani, Méghane S. Bataillard, A. Danese, Stacy Alves +7 more
2023bioRxiv22 citations Open access
Semantic Scholar
Sigma-1 Receptor Is Critical for Mitochondrial Activity and Unfolded Protein Response in Larval Zebrafish
Lucie Crouzier, Morgane Denus, Elodie M. Richard, Amarande Tavernier +4 more
2021International Journal of Molecular Sciences19 citations Open access
Semantic Scholar
Molecular analysis of the TMPRSS3 gene in Moroccan families with non-syndromic hearing loss.
M. Charif, O. Abidi, Redouane Boulouiz, Halima Nahili +5 more
2012Biochemical and Biophysical Research Communications - BBRC16 citations
Semantic Scholar
Improving mitochondria-associated endoplasmic reticulum membranes integrity as converging therapeutic strategy for rare neurodegenerative diseases and cancer.
Michal Cagalinec, M. Adnan, Silvia Borecka, G. Bultynck +17 more
2025Biochimica et biophysica acta. Molecular cell research15 citations Open access
Semantic Scholar
Wfs1E864K knock-in mice illuminate the fundamental role of Wfs1 in endocochlear potential production
Elodie M. Richard, E. Brun, Julia Korchagina, Lucie Crouzier +10 more
2023Cell Death and Disease15 citations Open access
Semantic Scholar
[FXYD proteins: novel regulators of Na,K-ATPase].
B. Delprat, S. Bibert, K. Geering
2006Medecine sciences : M/S14 citations Open access
Semantic Scholar
Dynamic expression of FXYD6 in the inner ear suggests a role of the protein in endolymph homeostasis and neuronal activity
B. Delprat, J. Puel, K. Geering
2007Developmental Dynamics13 citations Open access
Semantic Scholar
Calcium signaling and genetic rare diseases: An auditory perspective.
Elodie M. Richard, T. Maurice, B. Delprat
2023Cell Calcium12 citations Open access
Semantic Scholar
Amylovis-201 is a new dual-target ligand, acting as an anti-amyloidogenic compound and a potent agonist of the σ1 chaperone protein
Laura García-Pupo, Lucie Crouzier, Alberto Bencomo-Martínez, Johann Meunier +6 more
2024Acta Pharmaceutica Sinica B11 citations Open access
Semantic Scholar
Loss of Pde6a Induces Rod Outer Segment Shrinkage and Visual Alterations in pde6aQ70X Mutant Zebrafish, a Relevant Model of Retinal Dystrophy
Lucie Crouzier, Camille Diez, Elodie M. Richard, Nicolas Cubedo +5 more
2021Frontiers in Cell and Developmental Biology11 citations Open access
Semantic Scholar
Variants in USP48 encoding ubiquitin hydrolase are associated with autosomal dominant non-syndromic hereditary hearing loss.
S. Bassani, E. Beelen, M. Rossel, N. Voisin +22 more
2021Human Molecular Genetics10 citations Open access
Semantic Scholar
Convolamine, a tropane alkaloid extracted from Convolvulus plauricalis, is a potent sigma‐1 receptor‐positive modulator with cognitive and neuroprotective properties
Lucie Crouzier, Johann Meunier, Allison Carles, Axelle Morilleau +5 more
2023Phytotherapy Research9 citations Open access
Semantic Scholar
NCS1 overexpression restored mitochondrial activity and behavioral alterations in a zebrafish model of Wolfram syndrome
Lucie Crouzier, Elodie M. Richard, Camille Diez, Morgane Denus +6 more
2022Molecular Therapy: Methods & Clinical Development9 citations Open access
Semantic Scholar
Morphological, behavioral and cellular analyses revealed different phenotypes in Wolfram syndrome wfs1a and wfs1b zebrafish mutant lines
Lucie Crouzier, Elodie M. Richard, Camille Diez, Hala Alzaeem +9 more
2022Human Molecular Genetics9 citations Open access
Semantic Scholar
Defective Endoplasmic Reticulum–Mitochondria Connection Is a Hallmark of Wolfram Syndrome
B. Delprat, J. Rieusset, C. Delettre
2019Contact9 citations Open access
Semantic Scholar
Editorial: Organelles Relationships and Interactions: A Cancer Perspective
S. Patergnani, S. Marchi, B. Delprat, M. Wieckowski
2021Frontiers in Cell and Developmental Biology6 citations Open access
Semantic Scholar
Development of novel phenoxyalkylpiperidines as high-affinity Sigma-1 (σ1) receptor ligands with potent anti-amnesic effect.
F. S. Abatematteo, P. Mosier, M. Niso, Leonardo Brunetti +7 more
2021European journal of medicinal chemistry6 citations Open access
Semantic Scholar
Gene structure, chromosomal localization, and mutation screening of the human gene for the inner ear protein otospiralin
M. Lavigne-Rebillard, B. Delprat, M. Surget, J. Griffoin +4 more
2003Neurogenetics5 citations
Semantic Scholar
Sigma-1-targeting multimodal compound HBK-15 reverses memory deficits and restores hippocampal plasticity under NMDA hypofunction
Kinga Sałaciak, Klaudia Lustyk, Angelika Jagielska, M. Szafarz +13 more
2025Neurotherapeutics4 citations Open access
Semantic Scholar
Development of Second-Generation Phenoxyethylpiperidines as Potent Sigma-1 Receptor Agonists with Neuroprotective Potential for Alzheimer's Disease.
Francesco Mastropasqua, A. Lisi, Lucie Crouzier, Gabriella Rosanna Musillo +14 more
2026Journal of Medicinal Chemistry3 citations
Semantic Scholar
Positive modulation of sigma-1 receptor: a new weapon to mitigate disease progression in amyotrophic lateral sclerosis
Julien Le Friec, Hugo Mourier, S. Couly, N. Cubedo +10 more
2025Translational Neurodegeneration3 citations Open access
Semantic Scholar
Novel in vivo models of autosomal optic atrophy reveal conserved pathological changes in neuronal mitochondrial structure and function
Elin L Strachan, Eugène Dillon, Mairéad Sullivan, J. Glennon +6 more
2025The FASEB Journal2 citations Open access
Semantic Scholar
[Local administration of an AAV-TMPRSS3 vector in aged Tmprss3 mutant mice with progressive deafness restores hearing].
Elodie M. Richard, B. Delprat
2024Medecine sciences : M/S1 citations Open access
Semantic Scholar
WFS1E864K in humans and mice causes Wolfram-like syndrome optic atrophy via early axonal mitochondrial dysfunction
Hernán H. Dieguez, Kevin Dubois, É. Reboussin, C. de Muijnck +11 more
2025bioRxiv0 citations Open access
Semantic Scholar
ER-mitochondria crosstalk is regulated by NCS1 and is impaired in Wolfram syndrome
B. Delprat, C. Angebault, J. Fauconnier, S. Patergnani +7 more
2019Proceedings for Annual Meeting of The Japanese Pharmacological Society0 citations Open access
Semantic Scholar
Wolfram Syndrome murine models: phenotyping and gene therapy
Jolanta Jagodzinska, Delphine Bonnet-Wersinger, S. Kõks, B. Delprat +2 more
20160 citations
Semantic Scholar
P 413 Eye “degenerative evolution” in the Chinese cavefish: Triplophysa xiangxiensis Y.
N. Keller, J. Durand, G. Laumont, B. Delprat +1 more
19950 citations Open access
Semantic Scholar

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T. Maurice×13
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Elodie M. Richard×12
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Tangui Maurice — researcher profile on SciRah

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