Search Results - "Akil, Omar"

Astrocyte-derived interleukin-33 promotes microglial synapse engulfment and neural circuit development by Vainchtein, Ilia D, Chin, Gregory, Cho, Frances S, Kelley, Kevin W, Miller, John G, Chien, Elliott C, Liddelow, Shane A, Nguyen, Phi T, Nakao-Inoue, Hiromi, Dorman, Leah C, Akil, Omar, Joshita, Satoru, Barres, Ben A, Paz, Jeanne T, Molofsky, Ari B, Molofsky, Anna V

“…Neuronal synapse formation and remodeling are essential to central nervous system (CNS) development and are dysfunctional in neurodevelopmental diseases…”
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Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model by Akil, Omar, Dyka, Frank, Calvet, Charlotte, Emptoz, Alice, Lahlou, Ghizlene, Nouaille, Sylvie, de Monvel, Jacques Boutet, Hardelin, Jean-Pierre, Hauswirth, William W., Avan, Paul, Petit, Christine, Safieddine, Saaid, Lustig, Lawrence R.

“…Autosomal recessive genetic forms (DFNB) account for most cases of profound congenital deafness. Adeno-associated virus (AAV)-based gene therapy is a promising…”
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Restoration of Hearing in the VGLUT3 Knockout Mouse Using Virally Mediated Gene Therapy by Akil, Omar, Seal, Rebecca P., Burke, Kevin, Wang, Chuansong, Alemi, Aurash, During, Matthew, Edwards, Robert H., Lustig, Lawrence R.

“…Mice lacking the vesicular glutamate transporter-3 (VGLUT3) are congenitally deaf due to loss of glutamate release at the inner hair cell afferent synapse…”
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Deletion of Tmtc4 activates the unfolded protein response and causes postnatal hearing loss by Li, Jiang, Akil, Omar, Rouse, Stephanie L, McLaughlin, Conor W, Matthews, Ian R, Lustig, Lawrence R, Chan, Dylan K, Sherr, Elliott H

“…Hearing loss is a significant public health concern, affecting over 250 million people worldwide. Both genetic and environmental etiologies are linked to…”
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Sensorineural Deafness and Seizures in Mice Lacking Vesicular Glutamate Transporter 3 by Seal, Rebecca P., Akil, Omar, Yi, Eunyoung, Weber, Christopher M., Grant, Lisa, Yoo, Jong, Clause, Amanda, Kandler, Karl, Noebels, Jeffrey L., Glowatzki, Elisabeth, Lustig, Lawrence R., Edwards, Robert H.

“…The expression of unconventional vesicular glutamate transporter VGLUT3 by neurons known to release a different classical transmitter has suggested novel roles…”
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Modeling and Preventing Progressive Hearing Loss in Usher Syndrome III by Geng, Ruishuang, Omar, Akil, Gopal, Suhasini R., Chen, Daniel H.-C., Stepanyan, Ruben, Basch, Martin L., Dinculescu, Astra, Furness, David N., Saperstein, David, Hauswirth, William, Lustig, Lawrence R., Alagramam, Kumar N.

“…Usher syndrome type III (USH3) characterized by progressive loss of vision and hearing is caused by mutations in the clarin-1 gene ( CLRN1 ). Clrn1 knockout…”
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End-Stage Achalasia With Megaesophagus Refractory to Two Heller Myotomies by Chong Tai, Lorraine I, Akil, Omar, Nguyen, Kimberly Q, Sharma, Aryama

“…Achalasia is a motility disorder of the esophagus in which the lower esophageal sphincter fails to relax. Megaesophagus is a rare complication of achalasia…”
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Spiral ganglion degeneration and hearing loss as a consequence of satellite cell death in saposin B-deficient mice by Akil, Omar, Sun, Ying, Vijayakumar, Sarath, Zhang, Wujuan, Ku, Tiffany, Lee, Chi-Kyou, Jones, Sherri, Grabowski, Gregory A, Lustig, Lawrence R

“…Saposin B (Sap B) is an essential activator protein for arylsulfatase A in the hydrolysis of sulfatide, a lipid component of myelin. To study Sap B's role in…”
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Dual and triple AAV delivery of large therapeutic gene sequences into the inner ear by Akil, Omar

“…Adeno-associated virus (AAV)-mediated gene therapy has evolved from the bench to the bedside, and is now considered the therapy of choice for certain inherited…”
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Disrupted bone remodeling leads to cochlear overgrowth and hearing loss in a mouse model of fibrous dysplasia by Akil, Omar, Hall-Glenn, Faith, Chang, Jolie, Li, Alfred, Chang, Wenhan, Lustig, Lawrence R, Alliston, Tamara, Hsiao, Edward C

“…Normal hearing requires exquisite cooperation between bony and sensorineural structures within the cochlea. For example, the inner ear secretes proteins such…”
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Trophic factors are essential for the survival of grafted oligodendrocyte progenitors and for neuroprotection after perinatal excitotoxicity by Hirose-Ikeda, Megumi, Chu, Brian, Zhao, Paul, Akil, Omar, Escalante, Elida, Vergnes, Laurent, Cepeda, Carlos, Espinosa-Jeffrey, Araceli

“…The consequences of neonatal white matter injury are devastating and represent a major societal problem as currently there is no cure. Prematurity, low weight…”
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Surgical method for virally mediated gene delivery to the mouse inner ear through the round window membrane by Akil, Omar, Rouse, Stephanie L, Chan, Dylan K, Lustig, Lawrence R

“…Gene therapy, used to achieve functional recovery from sensorineural deafness, promises to grant better understanding of the underlying molecular and genetic…”
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Severe vestibular dysfunction and altered vestibular innervation in mice lacking prosaposin by Akil, Omar, Lustig, Lawrence R.

“…► Prosaposin is expressed and localized in vestibular end-organs and Scarpa's ganglia. ► Prosaposin is important for the maintenance of normal vestibular…”
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Comparison of functional and morphologic characteristics of mice models of noise-induced hearing loss by Park, Shi-Nae, Back, Sang-A, Park, Kyoung-Ho, Seo, Jae-Hyun, Noh, He-Il, Akil, Omar, Lustig, Laurence R, Yeo, Sang Won

“…Abstract Objectives This study was conducted to compare morphologic and audiologic changes after noise exposure in two different strains of mice (CBA and C57)…”
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Mouse Auditory Brainstem Response Testing by Akil, Omar, Oursler, A E, Fan, Kevin, Lustig, Lawrence R

“…The auditory brainstem response (ABR) test provides information about the inner ear (cochlea) and the central pathways for hearing. The ABR reflects the…”
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Progressive Deafness and Altered Cochlear Innervation in Knock-Out Mice Lacking Prosaposin by Akil, Omar, Chang, Jolie, Hiel, Hakim, Kong, Jee-Hyun, Yi, Eunyoung, Glowatzki, Elisabeth, Lustig, Lawrence R

“…After a yeast two-hybrid screen identified prosaposin as a potential interacting protein with the nicotinic acetylcholine receptor (nAChR) subunit alpha10,…”
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Cochlear Gene Therapy by Lustig, Lawrence, Akil, Omar

“…Over 450 million people worldwide suffer from hearing loss, leading to an estimated economic burden of ∼$750 billion. The past decade has seen significant…”
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AAV-Mediated Gene Delivery to the Inner Ear by Akil, Omar, Lustig, Lawrence

“…Cochlear gene therapy has made tremendous strides over the past 5 years. The first study documenting successful restoration of congenital hearing loss using…”
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Mouse Cochlear Whole Mount Immunofluorescence by Omar Akil, Lawrence Lustig

“…This protocol comprises the entire process of immunofluorescence staining on mouse cochlea whole mount, starting from tissue preparation to the mounting of the…”
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Neurotrophin gene therapy to promote survival of spiral ganglion neurons after deafness by Leake, Patricia A., Akil, Omar, Lang, Hainan

“…Hearing impairment is a major health and economic concern worldwide. Currently, the cochlear implant (CI) is the standard of care for remediation of severe to…”
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