Neurobiology of Disease


Date: May 1, 2009 (Friday)
Time: 10:30 a.m.
Theme: Sensory Failure (Hearing loss)
Module: Overview
Session: Posing a critical question: Deafness genes and molecules
Instructor: Teresa Nicolson
Description: There has been a rapid increase in the identification of genes involved in normal hearing as well as mutant genes involved in hereditary hearing loss. Following a short introduction to set the context, we will discuss two recent articles in a journal club format.
Study questions:
  1. Draw an annotated cartoon of a hair cell and identify at least 5 sites at which mutations in molecules might lead to deafness.
  2. How might miRNA96 (Mencia et al and Lewis et al) lead to hearing loss?
  3. How do microRNAs affect gene expression?
  1. Gillespie PG (2006). Molecular biology of hearing and balance. In Basic Neurochemistry, 7th Ed., (GJ Siegel, RW Albers, ST Brady, DL Price, eds.), Elsevier Academic Press, 833-840.
    Adobe Acrobat
  2. Mencia A, Modamio-Hoybjor S, Redshaw N, Morin M, Mayo-Merino F, Olavarrieta L, Aguirre LA, del Castillo I, Steel KP, Dalmay T, Moreno F, Moreno-Pelayo MA (2009). Mutations in the seed region of human miR-96 are responsible for nonsyndromic progressive hearing loss. Nat Genet 41:609-613.
    Adobe Acrobat
  3. Lewis MA, Quint E, Glazier AM, Fuchs H, De Angelis MH, Langford C, van Dongen S, Abreu-Goodger C, Piipari M, Redshaw N, Dalmay T, Moreno-Pelayo MA, Enright AJ, Steel KP. (2009). An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice. Nat Genet 41:614-618
    Adobe Acrobat