A, Sample records from 3-year-old patient 4. B, Model fits to the a-wave equation 1 and C, b-wave equation 2 data. See the "Activation of Phototransuction in Rods" and the "Analysis of B Waves" subsections of the "Methods" section for equations 1 and 2, respectively. Rod photoresponse parameters, S the sensitivity parameter and R mp3 the amplitude of the saturated rod response and represents the number of channels in the plasma membrane of the photoreceptor , representing the activation of rod phototransduction in 13 patients with Smith-Lemli-Opitz syndrome SLOS and 26 healthy control subjects. The upper and lower limits of the 95th and 99th prediction intervals and the normal means are as indicated. PL indicates the upper and lower limits of the 95th and 99th prediction intervals.
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