The relation of rhodopsin and scotopic retinal sensitivity in sector retinitis pigmentosa.
We used reflection retinal densitometric, psychophysical, and electroretinographic techniques to study the scotopic retinal function of a mother and her three daughters who had clinical evidence of a sectoral type of retinitis pigmentosa. Retinal regions with, and those without, ophthalmoscopic signs of degeneration were investigated. During dark adaptation, the time courses of rhodopsin regeneration and recovery of scotopic sensitivity were similar to normal as was the relation of rhodopsin to scotopic threshold. In dark-adapted eyes, threshold increases were not proportional to rhodopsin loss. The results of psychophysical tests of background adaptation and temporal summation, and analysis of the relation of electroretinographic a- to b-wave amplitudes, led to the conclusion that abnormalities of photoreceptor cell function central to the rhodopsin-bearing outer segments accounted for the increased thresholds.
Fulton
A B
AB
Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts.
Hansen
R M
RM
D000328:Adult; D003623:Dark Adaptation; D003720:Densitometry; D004596:Electroretinography; D005260:Female; D006801:Humans; D009887:Ophthalmoscopy; D010786:Photoreceptor Cells / Q000503:physiopathology; D012038:Regeneration; D012160:Retina / Q000503:physiopathology*; D012162:Retinal Degeneration / Q000503:physiopathology; D012168:Retinal Pigments / Q000502:physiology*; D012174:Retinitis Pigmentosa / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D012243:Rhodopsin / Q000502:physiology*; D058609:Visual Field Tests
D012168:Retinal Pigments; D012243:Rhodopsin