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Volume 17, Number 21, Issue of November 1, 1997 pp. 8118-8128
Copyright ©1997 Society for Neuroscience

The Effect of Peripherin/rds Haploinsufficiency on Rod and Cone Photoreceptors

Received July 15, 1997; revised Aug. 5, 1997; accepted Aug. 11, 1997.

Tong Cheng¹, Neal S. Peachey^{3, 4}, Shihong Li², Yoshinobu Goto^{3, 4}, Yun Cao², and Muna I. Naash^{1, 2}

Departments of ¹ Genetics and ² Ophthalmology and Visual Sciences, University of Illinois at Chicago, College of Medicine, Chicago, Illinois 60612, ³ Hines Veterans Affairs Hospital, Hines, Illinois 60141, and ⁴ Department of Neurology, Stritch School of Medicine, Loyola University of Chicago, Maywood, Illinois 60153

Haploinsufficiency because of a null mutation in the gene encoding peripherin/rds has been thought to be the primary defect associated with the photoreceptor degeneration seen in the retinal degeneration slow (rds) mouse. We have compared the effects of this haploinsufficiency on rod and cone photoreceptors by measuring the levels of rod- and cone-specific gene expression, by determining the relative rates of rod and cone degeneration, and by electroretinography. These analyses were performed at ages before and after the onset of degeneration of the photoreceptor cells. The data were consistent in demonstrating that measures for cone photoreceptors are relatively spared in comparison to comparable measures for rod photoreceptors. Blue cones were retained in higher number than red/green cones for the first 3 months of the degeneration. Our results indicate that the haploinsufficiency present in rds/+ mice has a greater impact on the rod than on the cone photoreceptor, a finding that likely reflects the tight regulation of peripherin/rds and the need for two functional alleles to assemble the structure of the rod outer segment and/or differences between the ultrastructure of the rod and cone outer segments.

Key words: photoreceptors; retinal degeneration; outer segment; electroretinograms; peripherin/rds

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