title: Gene Expression by Mouse Inner Ear Hair Cells during Development.
abstract: Hair cells of the inner ear are essential for hearing and balance. As a consequence, pathogenic variants in genes specifically expressed in hair cells often cause hereditary deafness. Hair cells are few in number and not easily isolated from the adjacent supporting cells, so the biochemistry and molecular biology of hair cells can be difficult to study. To study gene expression in hair cells, we developed a protocol for hair cell isolation by FACS. With nearly pure hair cells and surrounding cells, from cochlea and utricle and from E16 to P7, we performed a comprehensive cell type-specific RNA-Seq study of gene expression during mouse inner ear development. Expression profiling revealed new hair cell genes with distinct expression patterns: some are specific for vestibular hair cells, others for cochlear hair cells, and some are expressed just before or after maturation of mechanosensitivity. We found that many of the known hereditary deafness genes are much more highly expressed in hair cells than surrounding cells, suggesting that genes preferentially expressed in hair cells are good candidates for unknown deafness genes.
authors.affiliation: Department of Neurobiology and Howard Hughes Medical Institute, Harvard Medical School, Boston, Massachusetts 02115, Department of Otology and Laryngology, Harvard Medical School and Massachusetts Eye and Ear, Boston, Massachusetts 02114, and.
authors.forename: Déborah I
authors.identifier: http://orcid.org/0000-0002-6066-5907
authors.initials: DI
authors.lastname: Scheffer
authors.affiliation: Department of Neurobiology and Howard Hughes Medical Institute, Harvard Medical School, Boston, Massachusetts 02115, Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115.
authors.forename: Jun
authors.identifier: http://orcid.org/0000-0001-8362-7535
authors.initials: J
authors.lastname: Shen
authors.affiliation: Department of Neurobiology and Howard Hughes Medical Institute, Harvard Medical School, Boston, Massachusetts 02115, dcorey@hms.harvard.edu zheng-yi_chen@meei.harvard.edu.
authors.forename: David P
authors.identifier: http://orcid.org/0000-0003-4497-6016
authors.initials: DP
authors.lastname: Corey
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School and Massachusetts Eye and Ear, Boston, Massachusetts 02114, and dcorey@hms.harvard.edu zheng-yi_chen@meei.harvard.edu.
authors.forename: Zheng-Yi
authors.identifier: http://orcid.org/0000-0002-1452-4193
authors.initials: ZY
authors.lastname: Chen
mesh_terms: D000818:Animals; D002469:Cell Separation; D005434:Flow Cytometry; D020869:Gene Expression Profiling; D018507:Gene Expression Regulation, Developmental; D006199:Hair Cells, Auditory, Inner / Q000166:cytology / Q000378:metabolism*; D051379:Mice; D008822:Mice, Transgenic; D012444:Saccule and Utricle / Q000166:cytology / Q000254:growth & development / Q000378:metabolism
keywords: FACS; RNA-Seq; cochlea; development; hair cell; vestibule