With the development of the social economy, we are exposed to increasing noise in our daily lives. Our previous work found an ABCC1(NM_004996.3:c.A1769G, NP_004987.2:p.N590S) variant which cosegregated with the patients in an autosomal dominant non-syndromic hearing loss family. At present, the specific mechanism of deafness caused by ABCC1 mutation is still not clear. Using the knock-in mouse model simulating human ABCC1 mutation, we found that the occurrence of family-related phenotypes was likely attributed to the combination of the mouse genotype and low-intensity noise. GSH and GSSG are important physiological substrates of ABCC1. The destruction of GSH-GSSG balance in the cochleae of both Abcc1 N591S/+ mice and Abcc1 N591S/N591S mice during low-intensity noise exposure may result in irreversible damage to the hair cells of the cochleae, consequently leading to hearing loss in mice. The findings offered a potential novel idea for the prevention and management of hereditary hearing loss within this family.
Keyphrases
- hearing loss
- late onset
- air pollution
- high fat diet induced
- mouse model
- end stage renal disease
- newly diagnosed
- endothelial cells
- chronic kidney disease
- healthcare
- ejection fraction
- induced apoptosis
- oxidative stress
- mental health
- type diabetes
- autism spectrum disorder
- fluorescent probe
- physical activity
- climate change
- metabolic syndrome
- cell proliferation
- cell cycle arrest
- cell death
- skeletal muscle
- drug induced