Deafness: from genetic architecture to gene therapy
Progress in deciphering the genetic architecture of human sensorineural hearing
impairment (SNHI) or loss, and multidisciplinary studies of mouse models, have led to the …
impairment (SNHI) or loss, and multidisciplinary studies of mouse models, have led to the …
Repair Schwann cell update: Adaptive reprogramming, EMT, and stemness in regenerating nerves
KR Jessen, P Arthur‐Farraj - Glia, 2019 - Wiley Online Library
Schwann cells respond to nerve injury by cellular reprogramming that generates cells
specialized for promoting regeneration and repair. These repair cells clear redundant …
specialized for promoting regeneration and repair. These repair cells clear redundant …
A synthetic AAV vector enables safe and efficient gene transfer to the mammalian inner ear
LD Landegger, B Pan, C Askew, SJ Wassmer… - Nature …, 2017 - nature.com
Efforts to develop gene therapies for hearing loss have been hampered by the lack of safe,
efficient, and clinically relevant delivery modalities,. Here we demonstrate the safety and …
efficient, and clinically relevant delivery modalities,. Here we demonstrate the safety and …
AAV-ie enables safe and efficient gene transfer to inner ear cells
F Tan, C Chu, J Qi, W Li, D You, K Li, X Chen… - Nature …, 2019 - nature.com
Hearing loss is the most common sensory disorder. While gene therapy has emerged as a
promising treatment of inherited diseases like hearing loss, it is dependent on the …
promising treatment of inherited diseases like hearing loss, it is dependent on the …
Tbx2 is a master regulator of inner versus outer hair cell differentiation
J García-Añoveros, JC Clancy, CZ Foo, I García-Gómez… - Nature, 2022 - nature.com
The cochlea uses two types of mechanosensory cell to detect sounds. A single row of inner
hair cells (IHCs) synapse onto neurons to transmit sensory information to the brain, and …
hair cells (IHCs) synapse onto neurons to transmit sensory information to the brain, and …
Ti3C2TxMXene Composite 3D Hydrogel Potentiates mTOR Signaling to Promote the Generation of Functional Hair Cells in Cochlea Organoids
Z Zhang, S Gao, YN Hu, X Chen, C Cheng… - Advanced …, 2022 - Wiley Online Library
Organoids have certain cellular composition and physiological features in common with real
organs, making them promising models of organ formation, function, and diseases …
organs, making them promising models of organ formation, function, and diseases …
New treatment options for hearing loss
U Müller, PG Barr-Gillespie - Nature reviews Drug discovery, 2015 - nature.com
Hearing loss is the most common form of sensory impairment in humans and affects more
than 40 million people in the United States alone. No drug-based therapy has been …
than 40 million people in the United States alone. No drug-based therapy has been …
Clonal expansion of Lgr5-positive cells from mammalian cochlea and high-purity generation of sensory hair cells
Death of cochlear hair cells, which do not regenerate, is a cause of hearing loss in a high
percentage of the population. Currently, no approach exists to obtain large numbers of …
percentage of the population. Currently, no approach exists to obtain large numbers of …
Cellular reprogramming with ATOH1, GFI1, and POU4F3 implicate epigenetic changes and cell-cell signaling as obstacles to hair cell regeneration in mature …
Reprogramming of the cochlea with hair-cell-specific transcription factors such as ATOH1
has been proposed as a potential therapeutic strategy for hearing loss. ATOH1 expression …
has been proposed as a potential therapeutic strategy for hearing loss. ATOH1 expression …
Generation of inner ear hair cells by direct lineage conversion of primary somatic cells
L Menendez, T Trecek, S Gopalakrishnan, L Tao… - elife, 2020 - elifesciences.org
The mechanoreceptive sensory hair cells in the inner ear are selectively vulnerable to
numerous genetic and environmental insults. In mammals, hair cells lack regenerative …
numerous genetic and environmental insults. In mammals, hair cells lack regenerative …