Figure 8.
Electroretinography shows hCAPN5R243L mice have a specific loss of the inner retinal cells and cone cell function. (A) ERG tracing of ADNIV patient shows reduction of b-wave relative to the a-wave (14). The scotopic b-wave was 83% of normal (normal scotopic b-wave was 450 ± 100 μV in this system). (B) The photopic ERG b-wave was ∼32.5% of normal (339 ± 85 μV). The calibration mark before stimulus shows 100 μV. (C) A representative ERG test on an 8 month-old hCAPN5R243L transgenic mouse (green) compared with a control mouse (blue) displays the frequently observed functional phenotype. hCAPN5R243L transgenic mice have a reduced scotopic maximal b-wave amplitude compared with control mice, with the a-wave remaining unchanged. (D) Photopic, cone-specific ERGs showed a reduction in the b-wave maximal amplitude in the hCAPN5R243L transgenic mice compared with controls. Such a pattern correlates to ADNIV patients.