title: The expression of NLRX1 in C57BL/6 mice cochlear hair cells: Possible relation to aging- and neomycin-induced deafness.
abstract: Nucleotide-binding domain and leucine-rich-repeat-containing family member X1 (NLRX1) is a cytoplasmic pattern recognition receptor that is predominantly located in mitochondria, which is tightly related to mitochondrial damage, reactive oxygen species (ROS) production, inflammation and apoptosis. The present study was designed to explore whether NLRX1 expresses in C57BL/6 mice cochlear hair cells and, if so, to investigate the possible correlations between NLRX1 and hearing. The location and dynamic expression of NLRX1 were investigated by immunofluorescence, real-time PCR and Western blotting. Hearing thresholds of C57BL/6 mice were measured by auditory brainstem response (ABR). Moreover, the downstream inflammatory and apoptotic pathways regulated by NLRX1 were examined in age-related and neomycin-induced hair cell damage. Data showed that NLRX1 expressed in cytoplasm of C57BL/6 cochlear hair cells, especially in the cilia, which were essential for sound sensation. The expression of NLRX1 in hair cells increased as the mice grew up, and, decreased as they aged. Additionally, the activated apoptotic JNK pathway was detected in 9-month old mice with worse-hearing and 3-month old mice treated with neomycin. Overall, results indicate that NLRX1 may relate to hair cell maturity, hearing formation and maintenance, and promote hair cell apoptosis through JNK pathway induced by aging and neomycin.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Department of Pathology and Pathophysiology, Shandong University, School of Medicine, Jinan 250012, PR China.
authors.forename: Qianqian
authors.initials: Q
authors.lastname: Yang
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Shandong Provincial Key Laboratory of Otology, Jinan 250021, PR China.
authors.forename: Gaoying
authors.initials: G
authors.lastname: Sun
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Department of Pathology and Pathophysiology, Shandong University, School of Medicine, Jinan 250012, PR China.
authors.forename: Zhixin
authors.initials: Z
authors.lastname: Cao
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Shandong Provincial Key Laboratory of Otology, Jinan 250021, PR China.
authors.forename: Haiyan
authors.initials: H
authors.lastname: Yin
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Shandong Provincial Key Laboratory of Otology, Jinan 250021, PR China.
authors.forename: Qi
authors.initials: Q
authors.lastname: Qi
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Department of Pathology and Pathophysiology, Shandong University, School of Medicine, Jinan 250012, PR China.
authors.forename: Jinghan
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Shandong Provincial Key Laboratory of Otology, Jinan 250021, PR China.
authors.forename: Wenwen
authors.initials: W
authors.lastname: Liu
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Shandong Provincial Key Laboratory of Otology, Jinan 250021, PR China.
authors.forename: Xiaohui
authors.initials: X
authors.lastname: Bai
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Shandong Provincial Key Laboratory of Otology, Jinan 250021, PR China. Electronic address: wang.hb7585@hotmail.com.
authors.forename: Haibo
authors.initials: H
authors.lastname: Wang
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Provincial Hospital Affiliated to Shandong University, Jinan 250021, PR China; Department of Pathology and Pathophysiology, Shandong University, School of Medicine, Jinan 250012, PR China; Shandong Provincial Key Laboratory of Otology, Jinan 250021, PR China. Electronic address: lijianfeng@hotmail.com.
authors.forename: Jianfeng
authors.initials: J
authors.lastname: Li
mesh_terms: D000375:Aging / Q000502:physiology*; D000818:Animals; D000831:Animals, Newborn; D000900:Anti-Bacterial Agents / Q000009:adverse effects*; D017209:Apoptosis; D003638:Deafness / Q000139:chemically induced / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem; D006198:Hair Cells, Auditory / Q000187:drug effects / Q000378:metabolism*; D008810:Mice, Inbred C57BL; D024101:Mitochondrial Proteins / Q000378:metabolism*; D009355:Neomycin / Q000009:adverse effects*
chemical_list: D000900:Anti-Bacterial Agents; D024101:Mitochondrial Proteins; C555655:NLRX1 protein, mouse; D009355:Neomycin
keywords: Apoptosis; C57BL/6 mice; Hair cells; NLRX1; Nucleotide-binding domain and leucine-rich-repeat-containing family member X1