April 2010
Volume 51, Issue 13
ARVO Annual Meeting Abstract  |   April 2010
Proteomic Analysis Reveals Downregulated Expression of Protective Proteins in the Ush1c216AA Knock-In Retina
Author Affiliations & Notes
  • J. J. Lentz
    Neuroscience Center, LSUHSC, New Orleans, Louisiana
  • H. E. Farris
    Neuroscience Center, LSUHSC, New Orleans, Louisiana
  • W. C. Gordon
    Ophthalmology & Neuroscience Center,
    LSU Health Sciences Center, New Orleans, Louisiana
  • N. G. Bazan
    Ophthal & Neuroscience,
    LSU Health Sciences Center, New Orleans, Louisiana
  • Footnotes
    Commercial Relationships  J.J. Lentz, None; H.E. Farris, None; W.C. Gordon, None; N.G. Bazan, None.
  • Footnotes
    Support  Foundation Fighting Blindness Grant TA-NP-0808-0463-LSUNO; NIH/NEI Grant EY05121; NIH Cobre Grant RR016816
Investigative Ophthalmology & Visual Science April 2010, Vol.51, 3656. doi:https://doi.org/
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      J. J. Lentz, H. E. Farris, W. C. Gordon, N. G. Bazan; Proteomic Analysis Reveals Downregulated Expression of Protective Proteins in the Ush1c216AA Knock-In Retina. Invest. Ophthalmol. Vis. Sci. 2010;51(13):3656. doi: https://doi.org/.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose: : Usher syndrome is the leading cause of combined deafness and blindness. Patients with Usher I suffer retinitis pigmentosa beginning in early adolescence with congenital deafness and vestibular areflexia. The Ush1c216AA knock-in mice show a similar phenotype with retinal degeneration that begins after deafness. Abnormal electroretinograms in the mutant mice are evident as early as 1 month of age, however, the loss of rod photoreceptors begins between 6 and 12 months of age. Absent auditory-evoked brainstem responses between 3 weeks and 1 month of age indicate deafness at birth. The purpose of this study is to examine the retinal proteome in our knock-in mouse model containing the human USH1C216G>A mutation (216AA) over a one year time course.

Methods: : Pooled retinas from three 216AA mutant males or females were compared with three pooled, age and sex-matched wild type controls, respectively, using 2-dimensional fluorescence differential gel electrophoresis followed by protein identification by matrix-assisted laser-desorption ionization time-of-flight mass spectrometry.

Results: : At one year of age, there were 123 mutant protein spots that differed from their wild type age-and sex-matched littermates. All 123 spots were decreased in the mutant mice between 2 and 21 fold. Proteins decreased in the mutant belong to a diverse range of networks including intracellular transport, stress-response, and lipid metabolism. The protein spots with the highest fold changes (14 - 21 fold) are considered to be in the family of molecular chaperones with cell protective functions.

Conclusions: : Ush1c216AA

Keywords: retinal degenerations: cell biology • proteomics • retina 

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