Localization and expression of clarin-1, the Clrn1 gene product, in auditory hair cells and photoreceptors

Marisa Zallocchi, Daniel T. Meehan, Duane Delimont, Charles Askew, Suneetha Garige, Michael Anne Gratton, Christie A. Rothermund-Franklin, Dominic Cosgrove

Research output: Contribution to journalArticle

33 Scopus citations

Abstract

The Usher syndrome 3A (CLRN1) gene encodes clarin-1, which is a member of the tetraspanin family of transmembrane proteins. Although identified more than 6 years ago, little is known about its localization or function in the eye and ear. We developed a polyclonal antibody that react with all clarin-1 isoforms and used it to characterize protein expression in cochlea and retina. In the cochlea, we observe clarin-1expression in the stereocilia of P0 mice, and in synaptic terminals present at the base of the auditory hair cells from E18 to P6. In the retina, clarin-1 localizes to the connecting cilia, inner segment of photoreceptors and to the ribbon synapses. RT-PCR from P0 cochlea and P28 retina show mRNAs encoding only isoforms 2 and 3. Western blots show that only isoform 2 is present in protein extracts from these same tissues. We examined clarin-1 expression in the immortomouse-derived hair cell line UB/OC-1. Only isoform 2 is expressed in UB/OC-1 at both mRNA and protein levels, suggesting this isoform is biologically relevant to hair cell function. The protein co-localizes with microtubules and post-transgolgi vesicles. The subcellular localization of clarin-1 in hair cells and photoreceptors suggests it functions at both the basal and apical poles of neurosensoriepithelia.

Original languageEnglish (US)
Pages (from-to)109-120
Number of pages12
JournalHearing Research
Volume255
Issue number1-2
DOIs
StatePublished - Sep 2009

Keywords

  • Clarin-1
  • Connecting cilia
  • Ribbon synapse
  • Stereocilia
  • Usher syndrome

ASJC Scopus subject areas

  • Sensory Systems

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    Zallocchi, M., Meehan, D. T., Delimont, D., Askew, C., Garige, S., Gratton, M. A., Rothermund-Franklin, C. A., & Cosgrove, D. (2009). Localization and expression of clarin-1, the Clrn1 gene product, in auditory hair cells and photoreceptors. Hearing Research, 255(1-2), 109-120. https://doi.org/10.1016/j.heares.2009.06.006