Deficiency of the ER-stress-regulator MANF triggers progressive outer hair cell death and hearing loss
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چکیده
منابع مشابه
Cochlear outer-hair-cell efferents and complex-sound-induced hearing loss: protective and opposing effects.
Centrifugal crossed and uncrossed medial olivocochlear systems (CMOCS and UMOCS) terminate on cochlear outer hair cells (OHCs) and exercise effects through a nicotinic cholinergic receptor. Hence their cochlear effects have not been differentiated. Recent work on protection from loud-sound-induced temporary threshold shifts (TTSs) in hearing sensitivity suggest the two OHC efferent systems may ...
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Bacterial infections represent a rapidly growing challenge to human health. Aminoglycosides are widely used broad-spectrum antibiotics, but they inflict permanent hearing loss in up to ~50% of patients by causing selective sensory hair cell loss. Here, we hypothesized that reducing aminoglycoside entry into hair cells via mechanotransducer channels would reduce ototoxicity, and therefore we syn...
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Methionine sulfoxide reductase B3 (MsrB3) is a protein repair enzyme that specifically reduces methionine-R-sulfoxide to methionine. A recent genetic study showed that the MSRB3 gene is associated with autosomal recessive hearing loss in human deafness DFNB74. However, the precise role of MSRB3 in the auditory system and the pathogenesis of hearing loss have not yet been determined. This work i...
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Cyclodextrins are sugar compounds that are increasingly finding medicinal uses due to their ability to complex with hydrophobic molecules. One cyclodextrin in particular, 2-hydroxypropyl-β-cyclodextrin (HPβCD), is used as a carrier to solubilize lipophilic drugs and is itself being considered as a therapeutic agent for treatment of Niemann-Pick Type C disease, due to its ability to mobilize cho...
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A knockin (KI) mouse expressing mutated prestinV499G/Y501H (499 prestin) was created to study cochlear amplification. Recordings from isolated outer hair cells (OHC) in this mutant showed vastly reduced electromotility and, as a consequence, reduced hearing sensitivity. Although 499 prestin OHCs were normal in stiffness and longer than OHCs lacking prestin, accelerated OHC death was unexpectedl...
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ژورنال
عنوان ژورنال: Cell Death & Disease
سال: 2020
ISSN: 2041-4889
DOI: 10.1038/s41419-020-2286-6