Characterization of Strip1 Expression in Mouse Cochlear Hair Cells

Abstract

Striatin-interacting protein 1 (Strip1) is a core component of the striatin interacting phosphatase and kinase (STRIPAK) complex, which is involved in embryogenesis and development, circadian rhythms, type 2 diabetes, and cancer progression. However, the expression and role of Strip1 in the mammalian cochlea remains unclear. Here we studied the expression and function of Strip1 in the mouse cochlea by using Strip1 knockout mice. We first found that the mRNA and protein expression of Strip1 increases as mice age starting from postnatal day (P) 3 and reaches its highest expression level at P30 and that the expression of Strip1 can be detected by immunofluorescent staining starting from P14 only in cochlear HCs, and not in supporting cells (SCs). Next, we crossed Strip1 heterozygous knockout (Strip +/−) mice to obtain Strip1 homozygous knockout (Strip1−/−) mice for studying the role of Strip1 in cochlear HCs. However, no Strip1−/− mice were obtained and the ratio of Strip +/− to Strip1+/+ mice per litter was about 2:1, which suggested that homozygous Strip1 knockout is embryonic lethal. We measured hearing function and counted the HC number in P30 and P60 Strip +/− mice and found that they had normal hearing ability and HC numbers compared to Strip1+/+ mice. Our study suggested that Strip1 probably play important roles in HC development and maturation, which needs further study in the future.

Familial Temperature-Sensitive Auditory Neuropathy: Distinctive Clinical Courses Caused by Variants of the OTOF Gene

“…While supporting cells function as potential resources to regenerate HCs after damage (Lu et al, 2017;Zhang et al, 2017;Tan et al, 2019;Zhang S. et al, 2019Chen et al, 2021). Sensorineural hearing loss has many etiologies, varying from genetic defects (Zhu et al, 2018;Fang et al, 2019;Yang et al, 2019;Qian et al, 2020;Cheng et al, 2021;Fu et al, 2021a;Lv et al, 2021;Zhang et al, 2021) to environmental factors, including aging (Zhang et al, 2017;Fu et al, 2018;Li H. et al, 2018;He et al, 2020He et al, , 2021, noise and ototoxic drugs (Liu et al, 2016;He et al, 2017;Gao et al, 2020;Zhong et al, 2020;Fu et al, 2021b). TSAN is a rare phenotype with high clinical heterogeneity, manifesting as transient bilateral HL due to an increase in the core body temperature.…”

A Model of Waardenburg Syndrome Using Patient-Derived iPSCs With a SOX10 Mutation Displays Compromised Maturation and Function of the Neural Crest That Involves Inner Ear Development

“…Sound is collected and conducted by external and middle ear, then transformed into the electric signals by cochlear hair cells ( Wang et al, 2017 ; Liu Y. et al, 2019 ; Qi et al, 2019 , 2020 ; Zhang Y. et al, 2020 ); while spiral ganglion neurons is function as the neural auditory transduction cells ( Sun et al, 2016 ; Guo et al, 2019 , 2021 ; Liu W. et al, 2019 ; Zhao et al, 2019 ). The cochlear hair cells are sensitive to aging, acoustic trauma, ototoxic drugs, and environmental or genetic influences ( O’Donnell et al, 1988 ; Zhu et al, 2018 ; Fang et al, 2019 ; Jiang et al, 2020 ; Qian et al, 2020 ; Lv et al, 2021 ; Zhang et al, 2021 ). Previous studies have shown that oxidative stress and cell apoptosis play important roles in hair cell loss ( Sun et al, 2014 ; Yu et al, 2017 ; Li et al, 2018 ; Gao et al, 2019 ; Zhang Y. et al, 2019 ; Zhang et al, 2020b ; Zhong et al, 2020 ).…”

Disruption of Glutamate Release and Uptake-Related Protein Expression After Noise-Induced Synaptopathy in the Cochlea

“…The cochlear hair cells are sensitive to aging, acoustic trauma, ototoxic drugs, and environmental or genetic influences ( Zhu et al, 2018 ; Fang et al, 2019 ; He et al, 2020 ; Jiang et al, 2020 ; Qian et al, 2020 ; Lv et al, 2021 ; Zhang et al, 2021 ). Previous reports have shown that oxidative stress and cell apoptosis play important roles in noise induced hair cell loss and ribbon synaptopathy ( Sun et al, 2014 ; Yu et al, 2017 ; Li et al, 2018 ; Gao et al, 2019 ; He et al, 2019 ; Zhang Y. et al, 2019 ; Zhong et al, 2020 ).…”