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Cells of adult brain germinal zone have properties akin to hair cells and can be used to replace inner ear sensory cells after damage.

ABSTRACT: Auditory hair cell defect is a major cause of hearing impairment, often leading to spiral ganglia neuron (SGN) degeneration. The cell loss that follows is irreversible in mammals, because inner ear hair cells (HCs) have a limited capacity to regenerate. Here, we report that in the adult brain of both rodents and humans, the ependymal layer of the lateral ventricle contains cells with proliferative potential, which share morphological and functional characteristics with HCs. In addition, putative neural stem cells (NSCs) from the subventricular zone of the lateral ventricle can differentiate into functional SGNs. Also important, the NSCs can incorporate into the sensory epithelia, demonstrating their therapeutic potential. We assert that NSCs and edendymal cells can undergo an epigenetic functional switch to assume functional characteristics of HCs and SGNs. This study suggests that the functional plasticity of renewable cells and conditions that promote functional reprogramming can be used for cell therapy in the auditory setting.

SUBMITTER: Wei D

PROVIDER: S-EPMC2634930 | biostudies-literature | 2008 Dec

REPOSITORIES: biostudies-literature

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Cells of adult brain germinal zone have properties akin to hair cells and can be used to replace inner ear sensory cells after damage.

Wei Dongguang D Levic Snezana S Nie Liping L Gao Wei-qiang WQ Petit Christine C Jones Edward G EG Yamoah Ebenezer N EN

Proceedings of the National Academy of Sciences of the United States of America 20081208 52

Auditory hair cell defect is a major cause of hearing impairment, often leading to spiral ganglia neuron (SGN) degeneration. The cell loss that follows is irreversible in mammals, because inner ear hair cells (HCs) have a limited capacity to regenerate. Here, we report that in the adult brain of both rodents and humans, the ependymal layer of the lateral ventricle contains cells with proliferative potential, which share morphological and functional characteristics with HCs. In addition, putative ...[more]

PMID: 19064919

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Project description:The mammalian inner ear (IE) subserves auditory and vestibular sensations via highly specialized cells and proteins. Sensory receptor hair cells (HCs) are necessary for transducing mechanical inputs and stimulating sensory neurons by using a host of known and as yet unknown protein machinery. To understand the protein composition of these unique postmitotic cells, in which irreversible protein degradation or damage can lead to impaired hearing and balance, we analyzed IE samples by tandem mass spectrometry to generate an unbiased, shotgun-proteomics view of protein identities and abundances. By using Pou4f3/eGFP-transgenic mice in which HCs express GFP driven by Pou4f3, we FACS purified a population of HCs to analyze and compare the HC proteome with other IE subproteomes from sensory epithelia and whole IE. We show that the mammalian HC proteome comprises hundreds of uniquely or highly expressed proteins. Our global proteomic analysis of purified HCs extends the existing HC transcriptome, revealing previously undetected gene products and isoform-specific protein expression. Comparison of our proteomic data with mouse and human databases of genetic auditory/vestibular impairments confirms the critical role of the HC proteome for normal IE function, providing a cell-specific pool of candidates for novel, important HC genes. Several proteins identified exclusively in HCs by proteomics and verified by immunohistochemistry map to human genetic deafness loci, potentially representing new deafness genes. SIGNIFICANCE STATEMENT:Hearing and balance rely on specialized sensory hair cells (HCs) in the inner ear (IE) to convey information about sound, acceleration, and orientation to the brain. Genetically and environmentally induced perturbations to HC proteins can result in deafness and severe imbalance. We used transgenic mice with GFP-expressing HCs, coupled with FACS sorting and tandem mass spectrometry, to define the most complete HC and IE proteome to date. We show that hundreds of proteins are uniquely identified or enriched in HCs, extending previous gene expression analyses to reveal novel HC proteins and isoforms. Importantly, deafness-linked proteins were significantly enriched in HCs, suggesting that this in-depth proteomic analysis of IE sensory cells may hold potential for deafness gene discovery.

Dataset Information

Cells of adult brain germinal zone have properties akin to hair cells and can be used to replace inner ear sensory cells after damage.

Publications

Cells of adult brain germinal zone have properties akin to hair cells and can be used to replace inner ear sensory cells after damage.

Similar Datasets

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