Intracellular mechanisms in rd1 mouse retinal degeneration

Leif Johnson

Research output: ThesisDoctoral Thesis (compilation)


Retinitis pigmentosa (RP) is an inherited disorder and the leading cause of visual impairment in the working age population. It is caused by a number of different genetic mutations, all of which cause the rod photoreceptors to degenerate. As the rods become few in numbers, the cones will also begin to die, resulting in total blindness. Since the genetic defects leading to RP are numerous, it would be overwhelming to treat each variation individually. By finding mechanisms related to photoreceptor survival and degeneration which are common to all forms of RP, a more general therapy can be developed.

The rd1 mouse is an animal model of RP used to study these processes. Changes in retinal cells as a result of the rd1 mutation are characterized and manipulated using an in vitro system. Paper I describes how the signaling protein Akt is activated in correlation with developmental and pathological cell death and may be part of an endogenous survival program. Paper II investigates the retinal response to the CNTF and BDNF and shows how their own regulation is increased and the signaling proteins Akt, ERK and CREB are activated. Paper III illustrates the protective effects of antioxidant treatment on rd1 photoreceptors. Paper IV investigates the activation of JNK and c-Jun and evaluates their roles in rd1 degeneration. Paper V simulates the rd1 mutation pharmacologically at different ages and shows how intracellular mechanisms are affected by photoreceptor development. Characterizations such as these will help in the development of therapeutic strategies for RP.
Original languageEnglish
Awarding Institution
  • Ophthalmology, Lund
  • Ekström, Per, Supervisor
Award date2007 Sep 28
ISBN (Print)978-91-85897-07-0
Publication statusPublished - 2007

Bibliographical note

Defence details

Date: 2007-09-28
Time: 13:00
Place: Segerfalksalen BMC Hus A Sölvegatan 19 Lund

External reviewer(s)

Name: Karlsson, Jan-Olof
Title: Professor
Affiliation: Göteborg University


<div class="article_info">Leif E Johnson, Theo van Veen and Per A Ekstrom. <span class="article_issue_date">2005</span>. <span class="article_title">Differential Akt activation in the photoreceptors of normal and rd1 mice.</span> <span class="journal_series_title">Cell Tissue Res.</span>, <span class="journal_volume">vol 320</span> <span class="journal_pages">pp 213-22</span>.</div>
<div class="article_info">Seifollah Azadi, Leif E Johnson, Francois Paquet-Durand, Maithé T Perez, Yiqin Zhang, Per A Ekstrom and Theo van Veen. <span class="article_issue_date">2007</span>. <span class="article_title">CNTF+BDNF treatment and neuroprotective pathways in the rd1 mouse retina.</span> <span class="journal_series_title">Brain Res</span>, <span class="journal_volume">vol 1129</span> <span class="journal_pages">pp 116-29</span>.</div>
<div class="article_info">Maria M Sanz, Leif E Johnson, Satpal Ahuja, Per A Ekstrom, Javier Romero and Theo van Veen. <span class="article_issue_date">2007</span>. <span class="article_title">Significant photoreceptor rescue by treatment with a combination of antioxidants in an animal model for retinal degeneration.</span> <span class="journal_series_title">Neuroscience</span>, <span class="journal_volume">vol 145</span> <span class="journal_pages">pp 1120-9</span>.</div>
<div class="article_info">Leif E Johnson, Sandra Dahl, Theo van Veen and Per AR Ekström. <span class="article_issue_date">2007</span>. <span class="article_title">cJun signaling in degenerating photoreceptors of rd1 mice.</span> (submitted)</div>
<div class="article_info">Leif E Johnson and Per AR Ekström. <span class="article_issue_date">2007</span>. <span class="article_title">Developmental stage affects photoreceptor response in an induced model of retinitis pigmentosa.</span> (manuscript)</div>

Subject classification (UKÄ)

  • Ophthalmology


  • Ophtalmology
  • Neuroprotection
  • Photoreceptor
  • Retinitis Pigmentosa
  • Oftalmologi


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