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Rod Photoreceptor Temporal Properties in Retinal Degenerative Diseases

  • Yuquan WenEmail author
  • Kirsten G. Locke
  • Donald C. Hood
  • David G. Birch
Conference paper
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 723)

Abstract

Using paired-flash electroretinogram (ERG), our goal was to determine whether the inactivation of rod phototransduction is altered in patients with Retinal Degenerative Diseases (RDDs). The rod photoresponses were derived from 18 patients with autosomal dominant retinitis pigmentosa (adRP) (n = 18), 5 patients with cone–rod dystrophy (CRD), and 4 patients with Stargardt disease. Thirteen subjects with normal eye exams served as controls. T sat, the parameter describing phototransduction inactivation, was derived using the paired-flash ERG protocol. Rod a-wave recovery initiates at 544 ± 92 ms (mean ± SD) after a just-saturating test flash in subjects with normal vision. For patients with RDDs, the rod a-wave recovery initiates at 331 ± 99 ms (autosomal dominant RP, P < 0.001, t-test), 473 ± 113 ms (CRD, P = 0.26, t-test), and 491 ± 98 ms (Stargardt disease, P = 0.38, t-test). Thus patients with adRP show earlier-than-normal photoresponse recovery, while patients with CRD or Stargardt disease typically have T sat values within the normal range.

Keywords

Retinitis pigmentosa Cone–rod dystrophy Stargardt disease Retinal degeneration Electroretinography Phototransduction Rhodopsin Peripherin/rds ABCA4 

Notes

Acknowledgments

We thank Dr. Dianna Hughbanks-Wheaton and Kaylie Clark at the Southwest Eye Registry and the Daiger lab at UT Houston for coordinating and performing genetic testing. This investigation was supported by US National Institutes of Health grant (NEI R01 09076) to D.G.B and D.C.H. and the Foundation Fighting Blindness.

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Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Yuquan Wen
    • 1
    Email author
  • Kirsten G. Locke
    • 1
  • Donald C. Hood
    • 2
  • David G. Birch
    • 1
    • 3
  1. 1.Rose-Silverthorne Retinal Degenerations LaboratoryRetina Foundation of the SouthwestDallasUSA
  2. 2.Department of Psychology and OphthalmologyColumbia UniversityNew YorkUSA
  3. 3.Department of OphthalmologyUniversity of Texas Southwestern Medical CenterDallasUSA

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