{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T23:31:58Z","timestamp":1792279918668,"version":"4.4.0"},"reference-count":124,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2021,7,1]],"date-time":"2021-07-01T00:00:00Z","timestamp":1625097600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,7,1]],"date-time":"2021-07-01T00:00:00Z","timestamp":1625097600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,7,1]],"date-time":"2021-07-01T00:00:00Z","timestamp":1625097600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2021,7,1]],"date-time":"2021-07-01T00:00:00Z","timestamp":1625097600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2021,7,1]],"date-time":"2021-07-01T00:00:00Z","timestamp":1625097600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2021,7,1]],"date-time":"2021-07-01T00:00:00Z","timestamp":1625097600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2021,7,1]],"date-time":"2021-07-01T00:00:00Z","timestamp":1625097600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/501100000268","name":"Biotechnology and Biological Sciences Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000268","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Progress in Retinal and Eye Research"],"published-print":{"date-parts":[[2021,7]]},"DOI":"10.1016\/j.preteyeres.2020.100937","type":"journal-article","created":{"date-parts":[[2020,12,31]],"date-time":"2020-12-31T15:29:43Z","timestamp":1609428583000},"page":"100937","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":4,"special_numbering":"C","title":["Clinical vision and molecular loss: Integrating visual psychophysics with molecular genetics reveals key details of normal and abnormal visual processing"],"prefix":"10.1016","volume":"83","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9807-6289","authenticated-orcid":false,"given":"Andrew","family":"Stockman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. Bruce","family":"Henning","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew T.","family":"Rider","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.preteyeres.2020.100937_bib1a","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1136\/bjo.68.2.69","article-title":"Supernormal scotopic ERG in cone dystrophy","volume":"68","author":"Alexander","year":"1984","journal-title":"Brit. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib1","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/79944","article-title":"OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28","volume":"26","author":"Alexander","year":"2000","journal-title":"Nat. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib2","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1146\/annurev.physiol.64.082701.102229","article-title":"G proteins and phototransduction","volume":"64","author":"Arshavsky","year":"2002","journal-title":"Annu. Rev. Physiol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib3","doi-asserted-by":"crossref","first-page":"1887","DOI":"10.1056\/NEJMoa1414221","article-title":"Long-term effect of gene therapy on Leber's congenital amaurosis","volume":"372","author":"Bainbridge","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.preteyeres.2020.100937_bib4","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1056\/NEJMoa0802268","article-title":"Effect of gene therapy on visual function in Leber's congenital amaurosis","volume":"358","author":"Bainbridge","year":"2008","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.preteyeres.2020.100937_bib5","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1016\/j.ajo.2014.05.034","article-title":"Early macular retinal ganglion cell loss in dominant optic atrophy: genotype-phenotype correlation","volume":"158","author":"Barboni","year":"2014","journal-title":"Am. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib6","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1113\/jphysiol.1976.sp011492","article-title":"Threshold setting by the surround of cat retinal ganglion cells","volume":"259","author":"Barlow","year":"1976","journal-title":"J. Physiol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib7","series-title":"Contrast Sensitivity of the Human Eye and its Effects on Image Quality","author":"Barten","year":"1999"},{"key":"10.1016\/j.preteyeres.2020.100937_bib8","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.39166","article-title":"S-cone photoreceptors in the primate retina are functionally distinct from L and M cones","volume":"8","author":"Baudin","year":"2019","journal-title":"eLife"},{"key":"10.1016\/j.preteyeres.2020.100937_bib9","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.preteyeres.2010.03.004","article-title":"The molecular basis of human retinal and vitreoretinal diseases","volume":"29","author":"Berger","year":"2010","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib10","series-title":"The Lag of Visual Sensation in its Relation to Wave Lengths and Intensity of Light","author":"Bills","year":"1920"},{"key":"10.1016\/j.preteyeres.2020.100937_bib11","doi-asserted-by":"crossref","first-page":"1283","DOI":"10.1167\/iovs.09-4366","article-title":"Deactivation mechanisms of rod phototransduction the cogan lecture","volume":"51","author":"Burns","year":"2010","journal-title":"Invest. Ophth. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib12","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1146\/annurev.neuro.24.1.779","article-title":"Activation, deactivation and adaptation in vertebrate photoreceptor cells","volume":"24","author":"Burns","year":"2001","journal-title":"Annu. Rev. Neurosci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib13","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1038\/nrneurol.2014.228","article-title":"Disturbed mitochondrial dynamics and neurodegenerative disorders","volume":"11","author":"Burte","year":"2015","journal-title":"Nat. Rev. Neurol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib14","doi-asserted-by":"crossref","first-page":"1138","DOI":"10.1001\/archopht.125.8.1138","article-title":"Bradyopsia in an asian man","volume":"125","author":"Cheng","year":"2007","journal-title":"Arch. Ophthalmol-Chic"},{"key":"10.1016\/j.preteyeres.2020.100937_bib15","doi-asserted-by":"crossref","first-page":"1333","DOI":"10.1136\/bjo.2007.134726","article-title":"The natural history of OPA1-related autosomal dominant optic atrophy","volume":"92","author":"Cohn","year":"2008","journal-title":"Brit. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib16","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1016\/S0031-8914(52)80230-7","article-title":"Experiments on flicker and some calculations on an electrical analogue of the foveal systems","volume":"18","author":"de Lange","year":"1952","journal-title":"Physica"},{"key":"10.1016\/j.preteyeres.2020.100937_bib17","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1364\/JOSA.48.000777","article-title":"Research into the dynamic nature of the human fovea-cortex systems with intermittent and modulated light. I. Attenuation characteristics with white and colored light","volume":"48","author":"de Lange","year":"1958","journal-title":"J. Opt. Soc. Am."},{"key":"10.1016\/j.preteyeres.2020.100937_bib18","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1364\/JOSA.51.000415","article-title":"Eye's response at flicker fusion to square-wave modulation of a test field surrounded by a large steady field of equal mean luminance","volume":"51","author":"de Lange","year":"1961","journal-title":"J. Opt. Soc. Am."},{"key":"10.1016\/j.preteyeres.2020.100937_bib19","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1038\/79936","article-title":"Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy","volume":"26","author":"Delettre","year":"2000","journal-title":"Nat. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib20","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1001\/archopht.119.11.1667","article-title":"Autosomal dominant cone and cone-rod dystrophy with mutations in the guanylate cyclase activator 1A gene-encoding guanylate cyclase activating protein-1","volume":"119","author":"Downes","year":"2001","journal-title":"Arch. Ophthalmol-Chic"},{"key":"10.1016\/j.preteyeres.2020.100937_bib21","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1038\/nature06150","article-title":"Light adaptation in cone vision involves switching between receptor and post-receptor sites","volume":"449","author":"Dunn","year":"2007","journal-title":"Nature"},{"key":"10.1016\/j.preteyeres.2020.100937_bib22","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1152\/physrev.2001.81.1.117","article-title":"Adaptation in vertebrate photoreceptors","volume":"80","author":"Fain","year":"2001","journal-title":"Physiol. Rev."},{"key":"10.1016\/j.preteyeres.2020.100937_bib23","doi-asserted-by":"crossref","first-page":"192","DOI":"10.2475\/ajs.s3-44.261.192","article-title":"Persistence of vision","volume":"44","author":"Ferry","year":"1892","journal-title":"Am. J. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib24","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1007\/BF02764303","article-title":"Cone dystrophy and supernormal dark-adapted b-waves in the electroretinogram","volume":"228","author":"Foerster","year":"1990","journal-title":"Graefes Arch. Clin. Exp. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib25","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/0042-6989(78)90161-X","article-title":"The effects of photopigment depletion on brightness and threshold","volume":"18","author":"Geisler","year":"1978","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib26","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1001\/archopht.1983.01040010718003","article-title":"Cone dystrophy, nyctalopia, and supernormal rod responses. A new retinal degeneration","volume":"101","author":"Gouras","year":"1983","journal-title":"Arch. Ophthalmol-Chic"},{"key":"10.1016\/j.preteyeres.2020.100937_bib27","doi-asserted-by":"crossref","first-page":"3711","DOI":"10.1016\/0042-6989(96)00073-9","article-title":"The enhanced S cone syndrome: an analysis of receptoral and post-receptoral changes","volume":"36","author":"Greenstein","year":"1996","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib28","doi-asserted-by":"crossref","first-page":"100844","DOI":"10.1016\/j.preteyeres.2020.100844","article-title":"Cell types and cell circuits in human and non-human primate retina","volume":"78","author":"Gr\u00fcnert","year":"2020","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib29","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1038\/ng1097-194","article-title":"Mutations in RPE65 cause autosomal recessive childhood-onset severe retinal dystrophy","volume":"17","author":"Gu","year":"1997","journal-title":"Nat. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib30","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1038\/72777","article-title":"Mutation of a nuclear receptor gene, NR2E3, causes enhanced S cone syndrome, a disorder of retinal cell fate","volume":"24","author":"Haider","year":"2000","journal-title":"Nat. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib31","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1002\/humu.20010","article-title":"Leber congenital amaurosis: comprehensive survey of the genetic heterogeneity, refinement of the clinical definition, and genotype-phenotype correlations as a strategy for molecular diagnosis","volume":"23","author":"Hanein","year":"2004","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.preteyeres.2020.100937_bib32","doi-asserted-by":"crossref","first-page":"2323","DOI":"10.1016\/j.ophtha.2007.04.057","article-title":"Six patients with bradyopsia (slow vision) - clinical features and course of the disease","volume":"114","author":"Hartong","year":"2007","journal-title":"Ophthalmology"},{"key":"10.1016\/j.preteyeres.2020.100937_bib33","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1016\/0042-6989(95)00174-3","article-title":"Sites of disease action in a retinal dystrophy with supernormal and delayed rod electroretinogram b-waves","volume":"36","author":"Hood","year":"1996","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib34","doi-asserted-by":"crossref","first-page":"1473","DOI":"10.1016\/0042-6989(95)98727-Q","article-title":"Enhanced S cone syndrome: evidence for an abnormally large number of S cones","volume":"35","author":"Hood","year":"1995","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib35","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1364\/JOSA.6.000343","article-title":"A theory of intermittent vision","volume":"6","author":"Ives","year":"1922","journal-title":"J. Opt. Soc. Am."},{"key":"10.1016\/j.preteyeres.2020.100937_bib36","first-page":"827","article-title":"SWS (blue) cone hypersensitivity in a newly identified retinal degeneration","volume":"31","author":"Jacobson","year":"1990","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib37","doi-asserted-by":"crossref","first-page":"1604","DOI":"10.1016\/S0161-6420(86)33522-X","article-title":"Automated light- and dark-adapted perimetry for evaluating retinitis pigmentosa","volume":"93","author":"Jacobson","year":"1986","journal-title":"Ophthalmology"},{"key":"10.1016\/j.preteyeres.2020.100937_bib38","doi-asserted-by":"crossref","DOI":"10.1016\/0002-9394(79)90565-8","article-title":"A clinicopathologic study of autosomal dominant optic atrophy","volume":"88","author":"Johnston","year":"1979","journal-title":"Am. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib39","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/BF01203284","article-title":"Cone dysfunction and supernormal scotopic electroretinogram with a high-intensity stimulus. A report of three cases","volume":"84","author":"Kato","year":"1993","journal-title":"Doc. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib40","first-page":"425","article-title":"Enhanced S cone sensitivity syndrome: long-term follow-up, electrophysiological and psychophysical findings","volume":"8","author":"Kellner","year":"1993","journal-title":"Clin. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib41","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1364\/JOSA.51.000422","article-title":"Visual responses to time-dependent stimuli I. Amplitude sensitivity measurements","volume":"51","author":"Kelly","year":"1961","journal-title":"J. Opt. Soc. Am."},{"key":"10.1016\/j.preteyeres.2020.100937_bib42","article-title":"Infantile optic atrophy with dominant mode of inheritance: a clinical and genetic study of 19 Danish families","volume":"164","author":"Kjer","year":"1959","journal-title":"Acta Ophthalmol. Suppl."},{"key":"10.1016\/j.preteyeres.2020.100937_bib43","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1111\/j.1755-3768.1983.tb01424.x","article-title":"Histopathology of eye, optic nerve and brain in a case of dominant optic atrophy","volume":"61","author":"Kjer","year":"1983","journal-title":"Acta Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib44","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/0042-6989(71)90206-9","article-title":"Some stimulus parameters affecting spatial and temporal resolution of human vision","volume":"11","author":"Kulikowski","year":"1971","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib45","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1016\/j.preteyeres.2004.03.001","article-title":"Dark adaptation and the retinoid cycle of vision","volume":"23","author":"Lamb","year":"2004","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib46","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1038\/eye.1998.139","article-title":"Molecular basis of dark adaptation in rod photoreceptors","volume":"12","author":"Leibrock","year":"1998","journal-title":"Eye"},{"key":"10.1016\/j.preteyeres.2020.100937_bib47","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1090\/qam\/10666","article-title":"A method for the solution of certain non-linear problems in least squares","volume":"2","author":"Levenberg","year":"1944","journal-title":"Q. Appl. Math."},{"key":"10.1016\/j.preteyeres.2020.100937_bib48","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1146\/annurev.ps.29.020178.003145","article-title":"Visual sensitivity","volume":"29","author":"MacLeod","year":"1978","journal-title":"Annu. Rev. Psychol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib49","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1167\/iovs.16-20309","article-title":"The pattern of retinal ganglion cell loss in OPA1-related autosomal dominant optic atrophy inferred from temporal, spatial, and chromatic sensitivity losses","volume":"58","author":"Majander","year":"2017","journal-title":"Invest. Ophth. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib50","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1038\/ng1097-139","article-title":"Mutations in RPE65 cause Leber's congenital amaurosis","volume":"17","author":"Marlhens","year":"1997","journal-title":"Nat. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib51","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/S0002-9394(14)76980-6","article-title":"Diagnostic findings of a new syndrome with night blindness, maculopathy, and enhanced S cone sensitivity","volume":"110","author":"Marmor","year":"1990","journal-title":"Am. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib52","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1137\/0111030","article-title":"An algorithm for least-squares estimation of nonlinear parameters","volume":"11","author":"Marquardt","year":"1963","journal-title":"J. Soc. Ind. Appl. Math."},{"key":"10.1016\/j.preteyeres.2020.100937_bib53","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1364\/JOSA.58.000404","article-title":"Critical duration, the differential luminance threshold, critical flicker frequency, and visual adaptation: a theoretical treatment","volume":"58","author":"Matin","year":"1968","journal-title":"J. Opt. Soc. Am."},{"key":"10.1016\/j.preteyeres.2020.100937_bib54","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1038\/334067a0","article-title":"Photoreceptor light adaptation is mediated by cytoplasmic calcium concentration","volume":"334","author":"Matthews","year":"1988","journal-title":"Nature"},{"key":"10.1016\/j.preteyeres.2020.100937_bib55","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1016\/j.survophthal.2006.02.007","article-title":"Progressive cone and cone-rod dystrophies: phenotypes and underlying molecular genetic basis","volume":"51","author":"Michaelides","year":"2006","journal-title":"Surv. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib56","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1136\/bjo.2004.050567","article-title":"A detailed phenotypic study of \"cone dystrophy with supernormal rod ERG","volume":"89","author":"Michaelides","year":"2005","journal-title":"Brit. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib57","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1016\/j.ophtha.2009.06.011","article-title":"Novel mutations and electrophysiologic findings in RGS9-and R9AP-associated retinal dysfunction (bradyopsia)","volume":"117","author":"Michaelides","year":"2010","journal-title":"Ophthalmology"},{"key":"10.1016\/j.preteyeres.2020.100937_bib58","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1016\/j.ophtha.2005.02.024","article-title":"Mutation in the gene GUCA1A, encoding guanylate cyclase-activating protein 1, causes cone, cone-rod, and macular dystrophy","volume":"112","author":"Michaelides","year":"2005","journal-title":"Ophthalmology"},{"key":"10.1016\/j.preteyeres.2020.100937_bib59","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1073\/pnas.022533099","article-title":"The nuclear receptor NR2E3 plays a role in human retinal photoreceptor differentiation and degeneration","volume":"99","author":"Milam","year":"2002","journal-title":"Proc. Natl. Acad. Sci. Unit. States Am."},{"key":"10.1016\/j.preteyeres.2020.100937_bib60","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1111\/j.1755-3768.2008.01469.x","article-title":"Axonal loss occurs early in dominant optic atrophy","volume":"88","author":"Milea","year":"2010","journal-title":"Acta Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib61","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1038\/334069a0","article-title":"Calcium and light adaptation in retinal rods and cones","volume":"334","author":"Nakatani","year":"1988","journal-title":"Nature"},{"key":"10.1016\/j.preteyeres.2020.100937_bib62","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1038\/nature02170","article-title":"Defects in RGS9 or its anchor protein R9AP in patients with slow photoreceptor deactivation","volume":"427","author":"Nishiguchi","year":"2004","journal-title":"Nature"},{"key":"10.1016\/j.preteyeres.2020.100937_bib63","first-page":"286","article-title":"L'h\u00e9m\u00e9ralopie et les fonctions du pourple visuel","volume":"93","author":"Parinaud","year":"1881","journal-title":"Comptes rendus hebdomadaires des s\u00e9ances et m\u00e9mo. Soci. Biol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib64","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1093\/hmg\/7.2.273","article-title":"A mutation in guanylate cyclase activator 1A (GUCA1A) in an autosomal dominant cone dystrophy pedigree mapping to a new locus on chromosome 6p21.1","volume":"7","author":"Payne","year":"1998","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib65","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1016\/S1350-9462(98)00005-6","article-title":"Light adaptation and sensitivity controlling mechanisms in vertebrate photoreceptors","volume":"17","author":"Perlman","year":"1998","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib66","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1006\/mgme.1999.2906","article-title":"Leber congenital amaurosis","volume":"68","author":"Perrault","year":"1999","journal-title":"Mol. Genet. Metabol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib67","doi-asserted-by":"crossref","first-page":"1225","DOI":"10.1086\/302335","article-title":"Different functional outcome of retGC1 and RPE65 gene mutations in Leber congenital amaurosis","volume":"64","author":"Perrault","year":"1999","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib68","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1111\/j.1469-7793.2000.00591.x","article-title":"Characterisation of dark adaptation in human cone pathways: an application of the equivalent background hypothesis","volume":"528","author":"Pianta","year":"2000","journal-title":"J. Physiol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib69","doi-asserted-by":"crossref","first-page":"1384","DOI":"10.1016\/j.ophtha.2020.04.008","article-title":"Genetic basis of inherited retinal disease in a molecularly characterized cohort of more than 3000 families from the United Kingdom","volume":"127","author":"Pontikos","year":"2020","journal-title":"Ophthalmology"},{"key":"10.1016\/j.preteyeres.2020.100937_bib70","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1098\/rspl.1902.0032","article-title":"Contributions to the study of flicker. Paper II","volume":"70","author":"Porter","year":"1902","journal-title":"Proc. Roy. Soc. Lond."},{"key":"10.1016\/j.preteyeres.2020.100937_bib71","series-title":"Handbook of Biological Physics","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/S1383-8121(00)80008-1","article-title":"Phototransduction in vertebrate rods and cones: molecular mechanisms of amplification, recovery and light adaptation","volume":"vol. 3","author":"Pugh","year":"2000"},{"key":"10.1016\/j.preteyeres.2020.100937_bib72","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1016\/S0959-4388(99)80062-2","article-title":"Molecular mechanisms of vertebrate photoreceptor light adaptation","volume":"9","author":"Pugh","year":"1999","journal-title":"Curr. Opin. Neurobiol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib73","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1007\/BF01571319","article-title":"Die Stereskopie im Dienste der isochromen und heterochromen Photometrie","volume":"10","author":"Pulfrich","year":"1922","journal-title":"Naturwissenschaften"},{"key":"10.1016\/j.preteyeres.2020.100937_bib74","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1017\/S0952523800002789","article-title":"Light adaptation in the primate retina: analysis of changes in gain and dynamics of monkey retinal ganglion cells","volume":"4","author":"Purpura","year":"1990","journal-title":"Vis. Neurosci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib75","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1038\/3813","article-title":"Rpe65 is necessary for production of 11-cis-vitamin A in the retinal visual cycle","volume":"20","author":"Redmond","year":"1998","journal-title":"Nat. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib76","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0220358","article-title":"Light adaptation controls visual sensitivity by adjusting the speed and gain of the response to light","volume":"14","author":"Rider","year":"2019","journal-title":"PloS One"},{"key":"10.1016\/j.preteyeres.2020.100937_bib77","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1167\/iovs.13-12897","article-title":"Vision in observers with enhanced S-cone syndrome: an excess of S-cones but connected mainly to conventional S-cone pathways","volume":"55","author":"Ripamonti","year":"2014","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib78","doi-asserted-by":"crossref","first-page":"6817","DOI":"10.1167\/iovs.14-14923","article-title":"Nature of the visual loss in observers with Leber's congenital amaurosis caused by specific mutations in RPE65","volume":"55","author":"Ripamonti","year":"2014","journal-title":"Invest. Ophth. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib79","first-page":"51","article-title":"Cone dystrophy with supernormal rod electroretinogram: a comprehensive genotype\/phenotype study including fundus autofluorescence and extensive electrophysiology. Retina","volume":"30","author":"Robson","year":"2010","journal-title":"J. Retinal Vitreous Dis."},{"key":"10.1016\/j.preteyeres.2020.100937_bib80","doi-asserted-by":"crossref","first-page":"909","DOI":"10.1016\/0042-6989(72)90014-4","article-title":"Intensity versus adaptation and the Pulfrich stereophenomenon","volume":"12","author":"Rogers","year":"1972","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib81","doi-asserted-by":"crossref","first-page":"N51","DOI":"10.1088\/0967-3334\/28\/8\/N02","article-title":"Full-field stimulus testing (FST) to quantify visual perception in severely blind candidates for treatment trials","volume":"28","author":"Roman","year":"2007","journal-title":"Physiol. Meas."},{"key":"10.1016\/j.preteyeres.2020.100937_bib82","doi-asserted-by":"crossref","first-page":"2672","DOI":"10.1016\/j.ophtha.2013.08.008","article-title":"Imaging of the macula indicates early completion of structural deficit in autosomal-dominant optic atrophy","volume":"120","author":"Ronnback","year":"2013","journal-title":"Ophthalmology"},{"key":"10.1016\/j.preteyeres.2020.100937_bib83","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1111\/aos.12835","article-title":"Genotype-phenotype heterogeneity of ganglion cell and inner plexiform layer deficit in autosomal-dominant optic atrophy","volume":"93","author":"Ronnback","year":"2015","journal-title":"Acta Ophthalmol. Scand."},{"key":"10.1016\/j.preteyeres.2020.100937_bib84","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1111\/j.1755-3768.1993.tb04998.x","article-title":"Retinal cone dysfunction of supernormal rod ERG type. Five new cases","volume":"71","author":"Rosenberg","year":"1993","journal-title":"Acta Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib85","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/0042-6989(72)90118-6","article-title":"Dynamic properties of vision. II. Theoretical relationship between flicker and flash thresholds","volume":"12","author":"Roufs","year":"1972","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib86","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1016\/S0042-6989(98)00120-5","article-title":"The effects of temporal noise and retinal luminance on foveal flicker sensitivity","volume":"39","author":"Rovamo","year":"1999","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib87","doi-asserted-by":"crossref","first-page":"1099","DOI":"10.1016\/j.ajo.2008.02.004","article-title":"Novel KCNV2 mutations in cone dystrophy with supernormal rod electroretinogram","volume":"145","author":"Salah","year":"2008","journal-title":"Am. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib88","first-page":"2283","article-title":"Rod electroretinograms in an elevated cyclic guanosine monophosphate-type human retinal degeneration. Comparison with retinitis pigmentosa","volume":"31","author":"Sandberg","year":"1990","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib89","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1364\/JOSA.46.000721","article-title":"Optical and photoelectric analog of the eye","volume":"46","author":"Schade","year":"1956","journal-title":"J. Opt. Soc. Am."},{"key":"10.1016\/j.preteyeres.2020.100937_bib90","article-title":"Zur Anatomie und Physiologie der Retina","volume":"2","author":"Schultze","year":"1866","journal-title":"Archiv f\u00fcr mikroskopische Anatomie und Entwicklungsmechanik"},{"key":"10.1016\/j.preteyeres.2020.100937_bib91","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1016\/j.visres.2008.09.026","article-title":"Linear and nonlinear systems analysis of the visual system: why does it seem so linear? A review dedicated to the memory of Henk Spekreijse","volume":"49","author":"Shapley","year":"2009","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib92","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/0278-4327(84)90011-7","article-title":"Visual adaptation and retinal gain controls","volume":"3","author":"Shapley","year":"1984","journal-title":"Prog. Retin. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib93","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1001\/archopht.121.9.1316","article-title":"Shared mutations in NR2E3 in enhanced S-cone syndrome, Goldmann-Favre syndrome, and many cases of clumped pigmentary retinal degeneration","volume":"121","author":"Sharon","year":"2003","journal-title":"Arch. Ophthalmol-Chic"},{"key":"10.1016\/j.preteyeres.2020.100937_bib94","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1167\/tvst.5.3.10","article-title":"Selective automated perimetry under photopic, mesopic, and scotopic conditions: detection mechanisms and testing strategies","volume":"5","author":"Simunovic","year":"2016","journal-title":"Transl. Vis. Sci. Technol."},{"key":"10.1016\/j.preteyeres.2020.100937_bib95","doi-asserted-by":"crossref","first-page":"1133","DOI":"10.1364\/JOSA.58.001133","article-title":"Model for visual luminance discrimination and flicker detection","volume":"58","author":"Sperling","year":"1968","journal-title":"J. Opt. Soc. Am."},{"key":"10.1016\/j.preteyeres.2020.100937_bib96","series-title":"Report of a Joint Discussion on Vision Held on June 3, 1932, at the Imperial College of Science by the Physical and Optical Societies","first-page":"194","article-title":"Equivalent adaptation levels in localised retinal areas","author":"Stiles","year":"1932"},{"key":"10.1016\/j.preteyeres.2020.100937_bib97","series-title":"Handbook of Color Psychology","first-page":"27","article-title":"Fundamentals of color vision I: color processing in the eye","author":"Stockman","year":"2015"},{"key":"10.1016\/j.preteyeres.2020.100937_bib98","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1167\/iovs.13-12919","article-title":"Cone dystrophy with \u201csupernormal\u201d rod ERG: psychophysical testing shows comparable rod and cone temporal sensitivity losses with no gain in rod function","volume":"55","author":"Stockman","year":"2014","journal-title":"Invest. Ophth. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib99","doi-asserted-by":"crossref","first-page":"1930","DOI":"10.1167\/iovs.13-13682","article-title":"Visual consequences of molecular changes in the guanylate cyclase activating protein","volume":"55","author":"Stockman","year":"2014","journal-title":"Invest. Ophth. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib100","doi-asserted-by":"crossref","first-page":"1194","DOI":"10.1167\/6.11.5","article-title":"Human cone light adaptation: from behavioral measurements to molecular mechanisms","volume":"6","author":"Stockman","year":"2006","journal-title":"J. Vis."},{"key":"10.1016\/j.preteyeres.2020.100937_bib101","doi-asserted-by":"crossref","first-page":"1711","DOI":"10.1016\/S0042-6989(00)00021-3","article-title":"Spectral sensitivities of the middle- and long-wavelength sensitive cones derived from measurements in observers of known genotype","volume":"40","author":"Stockman","year":"2000","journal-title":"Vis. Res."},{"issue":"8","key":"10.1016\/j.preteyeres.2020.100937_bib102","doi-asserted-by":"crossref","DOI":"10.1167\/7.8.4","article-title":"The effect of sildenafil citrate (Viagra\u00ae) on visual sensitivity","volume":"7","author":"Stockman","year":"2007","journal-title":"J. Vis."},{"key":"10.1016\/j.preteyeres.2020.100937_bib103","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1167\/7.4.8","article-title":"Residual cone vision without \u03b1-transducin","volume":"7","author":"Stockman","year":"2007","journal-title":"J. Vis."},{"key":"10.1016\/j.preteyeres.2020.100937_bib104","doi-asserted-by":"crossref","DOI":"10.1167\/8.1.10","article-title":"The loss of the PDE6 deactivating enzyme, RGS9, results in precocious light adaptation at low light levels","volume":"8","author":"Stockman","year":"2008","journal-title":"J. Vis."},{"key":"10.1016\/j.preteyeres.2020.100937_bib105","doi-asserted-by":"crossref","first-page":"1992","DOI":"10.1364\/JOSAA.4.001992","article-title":"Temporal modulation sensitivity and pulse-detection thresholds for chromatic and luminance perturbations","volume":"4","author":"Swanson","year":"1987","journal-title":"J. Opt. Soc. Am. A"},{"key":"10.1016\/j.preteyeres.2020.100937_bib106","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1080\/13816810701503681","article-title":"Novel mutations in the KCNV2 gene in patients with cone dystrophy and a supernormal rod electroretinogram","volume":"28","author":"Thiagalingam","year":"2007","journal-title":"Ophthalmic Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib107","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/j.preteyeres.2012.04.003","article-title":"Lateral interactions in the outer retina","volume":"31","author":"Thoreson","year":"2012","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib108","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1038\/310314a0","article-title":"Retinal light adaptation - evidence for a feedback mechanism","volume":"310","author":"Tranchina","year":"1984","journal-title":"Nature"},{"key":"10.1016\/j.preteyeres.2020.100937_bib109","doi-asserted-by":"crossref","first-page":"3037","DOI":"10.1016\/0042-6989(95)00047-4","article-title":"Testing a computational model of light-adaptation dynamics","volume":"35","author":"von Wiegand","year":"1995","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib110","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1001\/archopht.116.3.351","article-title":"Clinical features in affected individuals from 21 pedigrees with dominant optic atrophy","volume":"116","author":"Votruba","year":"1998","journal-title":"Arch. Ophthalmol-Chic"},{"key":"10.1016\/j.preteyeres.2020.100937_bib111","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/j.preteyeres.2010.11.001","article-title":"The cone-specific visual cycle","volume":"30","author":"Wang","year":"2011","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib112","series-title":"Handbook of Perception and Human Performance","article-title":"Temporal sensitivity","author":"Watson","year":"1986"},{"key":"10.1016\/j.preteyeres.2020.100937_bib113","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1002\/sdtp.10196","article-title":"Extending the flicker visibility metric to a range of mean luminance","volume":"46","author":"Watson","year":"2015","journal-title":"SID Sympos. Digest Techn. Pap."},{"key":"10.1016\/j.preteyeres.2020.100937_bib114","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1016\/0042-6989(66)90078-2","article-title":"The Maxwellian view","volume":"6","author":"Westheimer","year":"1966","journal-title":"Vis. Res."},{"key":"10.1016\/j.preteyeres.2020.100937_bib115","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1167\/iovs.07-0471","article-title":"Cone dystrophy with supernormal rod response is strictly associated with mutations in KCNV2","volume":"49","author":"Wissinger","year":"2008","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib116","doi-asserted-by":"crossref","first-page":"3857","DOI":"10.1167\/iovs.03-1302","article-title":"Identification of RDH10, an all-trans retinol dehydrogenase, in retinal Muller cells","volume":"45","author":"Wu","year":"2004","journal-title":"Invest. Ophth. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib117","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1086\/507568","article-title":"Mutations in the gene KCNV2 encoding a voltage-gated Potassium channel subunit cause \u201ccone dystrophy with supernormal rod electroretinogram\u201d in humans","volume":"79","author":"Wu","year":"2006","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.preteyeres.2020.100937_bib118","first-page":"124","article-title":"ERG (electroretinogram) in hereditary optic atrophies","volume":"90","author":"Yagasaki","year":"1986","journal-title":"Nippon. Ganka Gakkai Zasshi"},{"key":"10.1016\/j.preteyeres.2020.100937_bib119","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1007\/BF00153014","article-title":"Two cases of retinal degeneration with an unusual form of electroretinogram","volume":"63","author":"Yagasaki","year":"1986","journal-title":"Doc. Ophthal."},{"key":"10.1016\/j.preteyeres.2020.100937_bib120","doi-asserted-by":"crossref","first-page":"1538","DOI":"10.1016\/j.ophtha.2009.12.038","article-title":"The prevalence and natural history of dominant optic atrophy due to OPA1 mutations","volume":"117","author":"Yu-Wai-Man","year":"2010","journal-title":"Ophthalmology"},{"key":"10.1016\/j.preteyeres.2020.100937_bib121","doi-asserted-by":"crossref","DOI":"10.3389\/fnmol.2018.00473","article-title":"The binding properties and physiological functions of Recoverin","volume":"11","author":"Zang","year":"2018","journal-title":"Front. Mol. Neurosci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib122","first-page":"1604","article-title":"Identification of the RPE65 protein in mammalian cone photoreceptors","volume":"43","author":"Znoiko","year":"2002","journal-title":"Invest. Ophth. Vis. Sci."},{"key":"10.1016\/j.preteyeres.2020.100937_bib123","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0046762","article-title":"Rod and cone function in patients with KCNV2 retinopathy","volume":"7","author":"Zobor","year":"2012","journal-title":"PloS One"}],"container-title":["Progress in Retinal and Eye Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1350946220301099?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1350946220301099?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T22:38:30Z","timestamp":1761172710000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1350946220301099"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7]]},"references-count":124,"alternative-id":["S1350946220301099"],"URL":"https:\/\/doi.org\/10.1016\/j.preteyeres.2020.100937","relation":{},"ISSN":["1350-9462"],"issn-type":[{"value":"1350-9462","type":"print"}],"subject":[],"published":{"date-parts":[[2021,7]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Clinical vision and molecular loss: Integrating visual psychophysics with molecular genetics reveals key details of normal and abnormal visual processing","name":"articletitle","label":"Article Title"},{"value":"Progress in Retinal and Eye Research","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.preteyeres.2020.100937","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 Elsevier Ltd. All rights reserved.","name":"copyright","label":"Copyright"}],"article-number":"100937"}}