Establishment of dog model of congenital stationary night blindness

• Project/Area Number: 26462683
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Ophthalmology
• Research Institution: Mie University
• Principal Investigator: Kondo Mineo 三重大学, 医学系研究科, 教授 (80303642)
• Co-Investigator(Kenkyū-buntansha): 杉本 昌彦 三重大学, 医学部附属病院, 講師 (00422874) ; 生杉 謙吾 三重大学, 医学系研究科, 講師 (10335135) ; 松原 央 三重大学, 医学部附属病院, 講師 (20378409)
• Project Period (FY): 2014-04-01 – 2017-03-31
• Project Status: Completed (Fiscal Year 2016)
• Budget Amount: ¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000); Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000); Fiscal Year 2015: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000); Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
• Keywords: 動物モデル / 網膜 / 夜盲 / 先天停在性夜盲 / 犬 / 網膜電図 / 遺伝 / ERG / 眼疾患 / 遺伝子 / 完全型 / CSNB / dog / 双極細胞 / 視細胞

Outline of Final Research Achievements

Congenital stationary night blindness (CSNB) is a non-progressive, clinically and genetically heterogeneous disease of impaired night vision. We report a naturally-occurring, stationary, autosomal recessive phenotype in beagle dogs with normal daylight vision but absent night vision. Affected dogs had normal retinas on clinical examination, but showed no detectable rod responses. They had “negative-type” mixed rod and cone responses in full-field ERGs. Their photopic long-flash ERGs had normal OFF-responses associated with severely reduced ON-responses. The phenotype is similar to the Schubert-Bornschein form of complete CSNB in humans. Genetic testing ruled out most known CSNB candidates. Retinal morphology showed normal cellular layers and structure, and electron microscopy revealed normal rod spherules and synaptic ribbons. Our results indicate involvement of a yet unknown gene in this canine model of complete CSNB.

Research Products

• [Int'l Joint Research] ペンシルバニア大学(米国)
• [Journal Article] Contributions of Second- and Third-Order Retinal Neurons to Cone Electroretinograms After Loss of Rod Function in Rhodopsin P347L Transgenic Rabbits (2017)
  • Author(s): Kominami T, Ueno S, Okado S, Nakanishi A, Kondo M, Terasaki H
  • Journal Title: Invest Ophthalmol Vis Sci Volume: 58 Issue: 3 Pages: 1417-1424
  • DOI: 10.1167/iovs.16-20344
  • Peer Reviewed / Open Access
• [Journal Article] A pharmacological approach in newly established retinal vein occlusion model (2017)
  • Author(s): Fuma S., Nishinaka A., Inoue Y., Tsuruma K., Shimazawa M., Kondo M. and Hara H
  • Journal Title: Scientific Reports Volume: 7, 43509 Issue: 1 Pages: 43509-43509
  • DOI: 10.1038/srep43509
  • Peer Reviewed / Open Access
• [Journal Article] Long-term Protection of Genetically-Ablated Rabbit Retinal Degeneration by Sustained Transscleral Unoprostone Delivery (2017)
  • Author(s): Nobuhiro Nagai, Eri Koyanagi, Yasuko Izumida, Junjun Liu, Aya Katsuyama, Hirokazu Kaji, Matsuhiko Nishizawa, Noriko Osumi, Mineo Kondo, Hiroko Terasaki, Yukihiko Mashima, Toru Nakazawa, Toshiaki Abe
  • Journal Title: Investigative Ophthalmology & Visual Science Volume: 57(15) Issue: 15 Pages: 6527-6538
  • DOI: 10.1167/iovs.16-20453
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Histopathological Changes of Inner Retina, Optic Disc, and Optic Nerve in Rabbit with Advanced Retinitis Pigmentosa (2016)
  • Author(s): Asakawa K, Ishikawa H, Uga S, Mashimo K, Kondo M, Terasaki H
  • Journal Title: Neuroophthalmology Volume: 40 Pages: 286-291
  • Peer Reviewed
• [Journal Article] Screening for diabetic retinopathy using new mydriasis-free, full-field flicker ERG recording device. (2016)
  • Author(s): Fukuo M, Kondo M, Hirose A, Fukushima H, Ikesugi K, Sugimoto M, Kato K, Uchigata Y, Kitano S.
  • Journal Title: Sci Rep. Volume: 8;6 Issue: 1 Pages: 36591-36591
  • DOI: 10.1038/srep36591
  • Peer Reviewed / Open Access
• [Journal Article] Method of Quantifying Size of Retinal Hemorrhages in Eyes with Branch Retinal Vein Occlusion Using 14-Square Grid: Interrater and Intrarater Reliability (2016)
  • Author(s): Takashima Y, Sugimoto M, Kato K, Kozawa M, Ikesugi K, Matsubara H, Kondo M
  • Journal Title: J Ophthalmol Volume: 2016 Pages: 1960190-1960190
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Novel RP1L1 variants and genotype-photoreceptor microstructural phenotype associations in cohort of Japanese patients with occult macular dystrophy. (2016)
  • Author(s): Fujinami K, Kameya S, Kikuchi S, Ueno S, Kondo M, Hayashi T, Shinoda K, Machida S, Kuniyoshi K, Kawamura Y, Akahori M, Yoshitake K, Katagiri S, Nakanishi A, Sakuramoto H, Ozawa Y, Tsubota K, Yamaki K, Mizota A, Terasaki H, Miyake Y, Iwata T, Tsunoda K.
  • Journal Title: Invest Ophthalmol Vis Sci. Volume: 57 Issue: 11 Pages: 4837-4846
  • DOI: 10.1167/iovs.16-19670
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Temporal Properties of Cone ERGs of Pikachurin Null Mutant Mouse (2016)
  • Author(s): Kominami T, Ueno S, Nakanishi A, Kominami A, Kondo M, Furukawa T, Terasaki H
  • Journal Title: Invest Ophthalmol Vis Sci Volume: 57 Pages: 1264-9
  • Peer Reviewed
• [Journal Article] Importance of Central Retinal Sensitivity for Prediction of Visual Acuity after Intravitreal Bevacizumb in Eyes with Macular Edema Associated with Branch Retinal Vein Occlusion (2016)
  • Author(s): Sugimoto M, Ichio A, Kondo M
  • Journal Title: PLoS One Volume: 10
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Effect of topical rebamipide on conjunctival goblet cell recovery after vitrectomy (2016)
  • Author(s): Kato K, Takashima Y, Matsunaga K, Sugimoto M, Matsubara H, Hirano K, Kondo M
  • Journal Title: Sci Rep Volume: 6 Issue: 1 Pages: 19516-19516
  • DOI: 10.1038/srep19516
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] A Naturally Occurring Canine Model of Autosomal Recessive Congenital Stationary Night Blindness. (2015)
  • Author(s): Mineo Kondo, Gautami Das, Ryoetsu Imai, Evelyn Santana, Tomio Nakashita, Miho Imawaka, Kosuke Ueda, Hirohiko Ohtsuka, Kazuhiko Sakai, Takehiro Aihara, Kumiko Kato, Masahiko Sugimoto, Shinji Ueno, Yuji Nishizawa, Gustavo D. Aguirre, Keiko Miyadera.
  • Journal Title: PloS One Volume: 10 Issue: 9 Pages: e0137072-e0137072
  • DOI: 10.1371/journal.pone.0137072
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Pathologic Changes of Cone Photoreceptors in Eyes With Occult Macular Dystrophy. (2015)
  • Author(s): Nakanishi A, Ueno S, Kawano K, Ito Y, Kominami T, Yasuda S, Kondo M, Tsunoda K, Iwata T, Terasaki H.
  • Journal Title: Invest Ophthalmol Vis Sci. Volume: Nov;56(12) Issue: 12 Pages: 7243-9
  • DOI: 10.1167/iovs.15-16742
  • Peer Reviewed / Open Access
• [Journal Article] Hidden Genetic Variation in LCA9-Associated Congenital Blindness Explained by 5'UTR Mutations and Copy-Number Variations of NMNAT1 (2015)
  • Author(s): Coppieters F, Todeschini AL, Fujimaki T, Baert A, De Bruyne M, Van Cauwenbergh C, Verdin H, Bauwens M, Ongenaert M, Kondo M, Meire F, Murakami A, Veitia RA, Leroy BP, De Baere E
  • Journal Title: Hum Mutat Volume: 36 Issue: 12 Pages: 1188-96
  • DOI: 10.1002/humu.22899
  • Peer Reviewed / Int'l Joint Research
• [Journal Article] Short Pulse Duration High-Power Laser Photocoagulation during Vitrectomy for Diabetic Retinopathy Reduces Postoperative Inflammation (2015)
  • Author(s): Sugimoto M, Ichio A, Kondo M
  • Journal Title: PLoS One Volume: 10 Issue: 8 Pages: e0135126-e0135126
  • DOI: 10.1371/journal.pone.0135126
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Exposure of haptic of posterior chamber intraocular lens after sutureless intrascleral fixation (2015)
  • Author(s): Matsui Y, Matsubara H, Hanemoto T, Kondo M
  • Journal Title: BMC Ophthalmol Volume: 15 Issue: 1 Pages: 104-104
  • DOI: 10.1186/s12886-015-0102-3
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Histopathologic findings of perforated corneas due to ferric ion infiltration (2015)
  • Author(s): Kato K, Hirano K, Takashima Y, Sakamoto S, Kondo M
  • Journal Title: Can J Ophthalmol Volume: 50 Issue: 4 Pages: 322-7
  • DOI: 10.1016/j.jcjo.2015.05.011
  • Peer Reviewed
• [Journal Article] Functional and morphological study of retinal photoreceptor cell degeneration in transgenic rabbits with a Pro347Leu rhodopsin mutation (2015)
  • Author(s): Asakawa K, Ishikawa H, Uga S, Mashimo K, Shimizu K, Kondo M, Terasaki H.
  • Journal Title: Jpn J Ophthalmol Volume: 59 Issue: 5 Pages: 353-63
  • DOI: 10.1007/s10384-015-0400-6
  • Peer Reviewed / Open Access
• [Journal Article] In vivo imaging of axonal transport of mitochondria in the diseased and aged mammalian CNS (2015)
  • Author(s): Takihara Y, Inatani M, Eto K, Inoue T, Kreymerman A, Miyake S, Ueno S, Nagaya M, Nakanishi A, Iwao K, Takamura Y, Sakamoto H, Satoh K, Kondo M, Sakamoto T, Goldberg JL, Nabekura J, Tanihara H
  • Journal Title: Proc Natl Acad Sci U S A Volume: 112 Pages: 10515-20
  • Peer Reviewed / Open Access
• [Journal Article] Significant Correlation between Retinal Venous Tortuosity and Aqueous Vascular Endothelial Growth Factor Concentration in Eyes with Central Retinal Vein Occlusion (2015)
  • Author(s): Yasuda S, Kachi S, Kondo M, Ueno S, Kaneko H, Terasaki H
  • Journal Title: PLoS One Volume: 10 Issue: 7 Pages: 0134267-0134267
  • DOI: 10.1371/journal.pone.0134267
  • Peer Reviewed / Open Access
• [Journal Article] Mutation analysis of BEST1 in Japanese patients with Best’s vitelliform macular dystrophy (2015)
  • Author(s): Katagiri S，Hayashi T，Ohkuma Y，Sekiryu T，Takeuchi T，Gekka T，et al.
  • Journal Title: Br J Ophthalmol Volume: 99 Issue: 11 Pages: 1577-1582
  • DOI: 10.1136/bjophthalmol-2015-306830
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Incidence of retinal detachment associated with atopic dermatitis in Japan: review of cases from 1992 to 2011 (2015)
  • Author(s): Sasoh M, Mizutani H, Matsubara H, Furuta M, Matsui Y, Yamanaka K, Kondo M
  • Journal Title: Clin Ophthalmol Volume: 9 Pages: 1129-34
  • DOI: 10.2147/opth.s85020
  • Peer Reviewed / Open Access
• [Journal Article] Pikachurin Protein Required for Increase of Cone Electroretinogram B-Wave during Light Adaptation (2015)
  • Author(s): Nagaya M, Ueno S, Kominami T, Nakanishi A, Koyasu T, Kondo M, Furukawa T, Terasaki H
  • Journal Title: PLoS One Volume: 10 Issue: 6 Pages: e0128921-e0128921
  • DOI: 10.1371/journal.pone.0128921
  • Peer Reviewed / Open Access
• [Journal Article] Effect of Pupil Size on Flicker ERGs Recorded With RETeval System: New Mydriasis-Free Full-Field ERG System. (2015)
  • Author(s): Kato K, Kondo M, Sugimoto M, Ikesugi K, Matsubara H
  • Journal Title: Invest Ophthalmol Vis Sci Volume: 56 Issue: 6 Pages: 3684-3690
  • DOI: 10.1167/iovs.14-16349
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Electroretinographic assessment of rod- and cone-mediated bipolar cell pathways using flicker stimuli in mice (2015)
  • Author(s): Tanimoto N, Sothilingam V, Kondo M, Biel M, Humphries P, Seeliger MW
  • Journal Title: Sci Rep Volume: 5 Issue: 1 Pages: 10731-10731
  • DOI: 10.1038/srep10731
  • Peer Reviewed / Open Access
• [Journal Article] A survey of the surgical treatment of congenital and developmental cataracts in Japan (2015)
  • Author(s): Nagamoto T, Oshika T, Fujikado T, Ishibashi T, Sato M, Kondo M, Kurosaka D, Azuma N
  • Journal Title: Jpn J Ophthalmol Volume: 59 Issue: 4 Pages: 203-8
  • DOI: 10.1007/s10384-015-0385-1
  • Peer Reviewed / Open Access
• [Journal Article] Clinical characteristics of congenital and developmental cataract undergoing surgical treatment (2015)
  • Author(s): Nagamoto T, Oshika T, Fujikado T, Ishibashi T, Sato M, Kondo M, Kurosaka D, Azuma N.
  • Journal Title: Jpn J Ophthalmol Volume: 59 Issue: 3 Pages: 148-56
  • DOI: 10.1007/s10384-015-0370-8
  • Peer Reviewed / Open Access
• [Journal Article] RPE65 Mutations in Two Japanese Families with Leber Congenital Amaurosis (2015)
  • Author(s): Katagiri S, Hayashi T, Kondo M, Tsukitome H, Yoshitake K, Akahori M, Ikeo K, Tsuneoka H, Iwata T
  • Journal Title: Ophthalmic Genet. 2015 Aug 21:1-9. Volume: 21 Pages: 1-9
  • Peer Reviewed
• [Journal Article] Clinical characteristics of congenital and developmental cataract undergoing surgical treatment. (2015)
  • Author(s): Nagamoto T, Oshika T, Fujikado T, Ishibashi T, Sato M, Kondo M, Kurosaka D, Azuma N.
  • Journal Title: Jpn J Ophthalmol. Volume: 未確定
  • Peer Reviewed
• [Journal Article] Transient increase of retinal nerve fiber layer thickness after macular hole surgery (2014)
  • Author(s): Hibi N, Kondo M, Ishikawa K, Ueno S, Komeima K, Terasaki H.
  • Journal Title: Int Ophthalmol. Volume: 34 Pages: 575-581
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Ultra-WideField fluorescein angiography by oral administration of fluorescein. (2014)
  • Author(s): Sugimoto M, Matsubara H, Miyata R, Matsui Y, Ichio A, Kondo M.
  • Journal Title: Acta Ophthalmol. Volume: 92 Issue: 5 Pages: 417-418
  • DOI: 10.1111/aos.12323
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Probing of congenital nasolacrimal duct obstruction with dacryoendoscope. (2014)
  • Author(s): Kato K, Matsunaga K, Takashima Y, Kondo M.
  • Journal Title: Clin Ophthalmol. Volume: 16 Pages: 977-980
  • DOI: 10.2147/opth.s60377
  • Peer Reviewed / Open Access
• [Journal Article] The first USH2A mutation analysis of Japanese autosomal recessive retinitis pigmentosa patients: a totally different mutation profile with the lack of frequent mutations found in Caucasian patients. (2014)
  • Author(s): Yang Zhao, Katsuhiro Hosono, Kimiko Suto, Chie Ishigami, Yuki Arai, Akiko Hikoya, Yasuhiko Hirami, Masafumi Ohtsubo, Shinji Ueno, Hiroko Terasaki, Miho Sato, Hiroshi Nakanishi, Shiori Endo, Kunihiro Mizuta, Hiroyuki Mineta, Mineo Kondo, Masayo Takahashi, Shinsei Minoshima, Yoshihiro Hotta.
  • Journal Title: J Hum Genet. Volume: 59 Issue: 9 Pages: 521-528
  • DOI: 10.1038/jhg.2014.65
  • NAID: 40020198536
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Electroretinograms and level of aqueous vascular endothelial growth factor in eyes with hemicentral retinal vein occlusion or branch retinal vein occlusion. (2014)
  • Author(s): Yasuda S, Kachi S, Ueno S, Ushida H, Piao CH, Kondo M, Terasaki H.
  • Journal Title: Jpn J Ophthalmol. Volume: 58 Issue: 3 Pages: 232-236
  • DOI: 10.1007/s10384-014-0316-6
  • Peer Reviewed
• [Journal Article] Changes in outer retinal microstructures during six month period in eyes with acute zonal occult outer retinopathy-complex. (2014)
  • Author(s): Matsui Y, Matsubara H, Ueno S, Ito Y, Terasaki H, Kondo M.
  • Journal Title: PLoS One Volume: 9 Issue: 10 Pages: e110592-e110592
  • DOI: 10.1371/journal.pone.0110592
  • Peer Reviewed / Open Access
• [Journal Article] Bilateral retinal detachment in Werner syndrome. (2014)
  • Author(s): Sasoh M, Tsukitome H, Matsui Y, Furuta M, Kondo M.
  • Journal Title: Retin Cases Brief Rep. Volume: 8 Issue: 2 Pages: 92-94
  • DOI: 10.1097/icb.0000000000000011
  • Peer Reviewed
• [Journal Article] RPE65 Mutations in Two Japanese Families with Leber Congenital Amaurosis. (2014)
  • Author(s): Katagiri S, Hayashi T, Kondo M, Tsukitome H, Yoshitake K, Akahori M, Ikeo K, Tsuneoka H, Iwata T.
  • Journal Title: Ophthalmic Genet. Volume: 12 Pages: 1-9
  • Peer Reviewed
• [Presentation] Topics in diagnosis of retinal diseases (2017)
  • Author(s): Mineo Kondo
  • Organizer: All India Ophthalmological Society
  • Place of Presentation: Jaipur, India
  • Year and Date: 2017-02-16
  • Int'l Joint Research / Invited
• [Presentation] Amplitude increase of flicker ERG in eyes with non-ischemiv CRVO (2016)
  • Author(s): Mineo Kondo
  • Organizer: International Society for Clinical Electrophysiology of Vision
  • Place of Presentation: Singapore
  • Year and Date: 2016-08-13
  • Int'l Joint Research
• [Presentation] Clinical application of multifocal ERG (2016)
  • Author(s): Mineo Kondo
  • Organizer: Association for Research in Vision and Ophthalmology
  • Place of Presentation: Seattle, USA
  • Year and Date: 2016-04-30
  • Int'l Joint Research / Invited
• [Presentation] Effect of pupil size on flicker ERGs Recorded with RETeval system: experiments using mydriatics and artificial pupils (2015)
  • Author(s): Kondo M, Kato K, Sugimoto M, Ikesugi K, Matsubara H
  • Organizer: 53rd Symposium of International Society for Clinical Electrophysiology of Vision
  • Place of Presentation: Ljubljana, Slovenia
  • Year and Date: 2015-06-25
  • Int'l Joint Research
• [Presentation] Clinical application and recent advances in electroretinography(ERG). (2014)
  • Author(s): Kondo M
  • Organizer: Combined AAPOS, JAPO, and JASA
  • Place of Presentation: Kyoto 京都国際会館
  • Year and Date: 2014-11-30
  • Invited
• [Presentation] Effect of pupil size on flicker ERGs recorded by new mydriasis-free ERG system (2014)
  • Author(s): Kondo M
  • Organizer: ISCEV
  • Place of Presentation: Boston, USA
  • Year and Date: 2014-07-23
• [Presentation] Importance, application and relevance of animal models to clinical human ERG. International Society for Clinical Electrophysiology of Vision. (2014)
  • Author(s): Kondo M
  • Organizer: ISCEV
  • Place of Presentation: Boston, USA
  • Year and Date: 2014-07-20
  • Invited
• [Presentation] Controversies in Retinal Vein Occlusion. (2014)
  • Author(s): Kondo M
  • Organizer: WOC 2014 Tokyo
  • Place of Presentation: Tokyo 国際フォーラム
  • Year and Date: 2014-04-02
  • Invited