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The model accurately reproduces the rod response when stimulated with a simulated photocurrent signal.
To study the clinical variability and KCNV2 mutation spectrum in cone dystrophy with supernormal rod response (CDSRR) in the Israeli population.
We chose this non-degenerate configuration such that the wavelength of idler and signal mode coincided with the maximum quantum efficiency of our custom-coated Andor iXon Ultra EMCCD camera and the peak of the human rod response, respectively.
Rather these data argue for a more specific involvement of Gnat2 in the Gnat1-independent rod response.
Furthermore, the association between Gnat2 and the Gnat1-independent rod response implied by our Gnat1−/− Gnat2cpfl3/cpfl3 data could, of course, be indirect.
As these match the sensory characteristics of the TKO ERG it seems likely that the visual responses we report here reflect the activity of this rare rod response.
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(B – C ) Rod and cone flashes that produced near-equal amplitude responses in RGCs elicited strong cone responses and weak rod responses in the axon terminals of L-cones (B ) and in horizontal cells (C ).
Rod responses to proteotoxicity correlate with the nature, level and ratio of mutant ATXN7 species.
What is happening is that, in this range of luminosities, called mesopic, both rods and cones are responding, and, as the rod responses become more pronounced i.e., as darkness increases the rod luminosity scale prevails over that of the cones.
Pd RBCs showed smaller dim flash responses and steeper intensity-response relationships than WT RBCs, consistent with the smaller rod responses being selectively filtered out by the non-linear threshold at the rod-to-rod bipolar synapse.
Full-field electroretinogram testing was normal for the right eye, with the exception of prolonged implicit time of the b-wave maximal response, and abnormal for the left eye, with reduced amplitudes of the isolated and maximal rod responses and a significantly prolonged 30 Hz cone flicker implicit time.
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