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Items: 1 to 50 of 62


Rescue of cone function in cone-only Nphp5 knockout mouse model with Leber congenital amaurosis phenotype.

Hanke-Gogokhia C, Chiodo VA, Hauswirth WW, Frederick JM, Baehr W.

Mol Vis. 2018 Dec 30;24:834-846. eCollection 2018.


Deletion of both centrin 2 (CETN2) and CETN3 destabilizes the distal connecting cilium of mouse photoreceptors.

Ying G, Frederick JM, Baehr W.

J Biol Chem. 2019 Mar 15;294(11):3957-3973. doi: 10.1074/jbc.RA118.006371. Epub 2019 Jan 15.


Insights into photoreceptor ciliogenesis revealed by animal models.

Baehr W, Hanke-Gogokhia C, Sharif A, Reed M, Dahl T, Frederick JM, Ying G.

Prog Retin Eye Res. 2019 Jul;71:26-56. doi: 10.1016/j.preteyeres.2018.12.004. Epub 2018 Dec 25. Review.


The small GTPase RAB28 is required for phagocytosis of cone outer segments by the murine retinal pigmented epithelium.

Ying G, Boldt K, Ueffing M, Gerstner CD, Frederick JM, Baehr W.

J Biol Chem. 2018 Nov 9;293(45):17546-17558. doi: 10.1074/jbc.RA118.005484. Epub 2018 Sep 18.


Supplementation with macular carotenoids improves visual performance of transgenic mice.

Li B, Rognon GT, Mattinson T, Vachali PP, Gorusupudi A, Chang FY, Ranganathan A, Nelson K, George EW, Frederick JM, Bernstein PS.

Arch Biochem Biophys. 2018 Jul 1;649:22-28. doi: 10.1016/ Epub 2018 May 6.


Binary Function of ARL3-GTP Revealed by Gene Knockouts.

Hanke-Gogokhia C, Frederick JM, Zhang H, Baehr W.

Adv Exp Med Biol. 2018;1074:317-325. doi: 10.1007/978-3-319-75402-4_39. Review.


The guanine nucleotide exchange factor Arf-like protein 13b is essential for assembly of the mouse photoreceptor transition zone and outer segment.

Hanke-Gogokhia C, Wu Z, Sharif A, Yazigi H, Frederick JM, Baehr W.

J Biol Chem. 2017 Dec 29;292(52):21442-21456. doi: 10.1074/jbc.RA117.000141. Epub 2017 Oct 31.


Small GTPases Rab8a and Rab11a Are Dispensable for Rhodopsin Transport in Mouse Photoreceptors.

Ying G, Gerstner CD, Frederick JM, Boye SL, Hauswirth WW, Baehr W.

PLoS One. 2016 Aug 16;11(8):e0161236. doi: 10.1371/journal.pone.0161236. eCollection 2016.


Ciliopathy-associated IQCB1/NPHP5 protein is required for mouse photoreceptor outer segment formation.

Ronquillo CC, Hanke-Gogokhia C, Revelo MP, Frederick JM, Jiang L, Baehr W.

FASEB J. 2016 Oct;30(10):3400-3412. Epub 2016 Jun 21.


Arf-like Protein 3 (ARL3) Regulates Protein Trafficking and Ciliogenesis in Mouse Photoreceptors.

Hanke-Gogokhia C, Wu Z, Gerstner CD, Frederick JM, Zhang H, Baehr W.

J Biol Chem. 2016 Mar 25;291(13):7142-55. doi: 10.1074/jbc.M115.710954. Epub 2016 Jan 25.


The Function of Arf-like Proteins ARL2 and ARL3 in Photoreceptors.

Hanke-Gogokhia C, Zhang H, Frederick JM, Baehr W.

Adv Exp Med Biol. 2016;854:655-61. doi: 10.1007/978-3-319-17121-0_87.


Kinesin family 17 (osmotic avoidance abnormal-3) is dispensable for photoreceptor morphology and function.

Jiang L, Tam BM, Ying G, Wu S, Hauswirth WW, Frederick JM, Moritz OL, Baehr W.

FASEB J. 2015 Dec;29(12):4866-80. doi: 10.1096/fj.15-275677. Epub 2015 Jul 30.


The effect of continuous interscalene brachial plexus block with 0.125% bupivacaine vs 0.2% ropivacaine on pain relief, diaphragmatic motility, and ventilatory function.

Choromanski DW, Patel PS, Frederick JM, Lemos SE, Chidiac EJ.

J Clin Anesth. 2015 Dec;27(8):619-26. doi: 10.1016/j.jclinane.2015.03.006. Epub 2015 Jul 26.


Heterotrimeric kinesin-2 (KIF3) mediates transition zone and axoneme formation of mouse photoreceptors.

Jiang L, Wei Y, Ronquillo CC, Marc RE, Yoder BK, Frederick JM, Baehr W.

J Biol Chem. 2015 May 15;290(20):12765-78. doi: 10.1074/jbc.M115.638437. Epub 2015 Mar 30.


Mistrafficking of prenylated proteins causes retinitis pigmentosa 2.

Zhang H, Hanke-Gogokhia C, Jiang L, Li X, Wang P, Gerstner CD, Frederick JM, Yang Z, Baehr W.

FASEB J. 2015 Mar;29(3):932-42. doi: 10.1096/fj.14-257915. Epub 2014 Nov 24.


Inactivity of human β,β-carotene-9',10'-dioxygenase (BCO2) underlies retinal accumulation of the human macular carotenoid pigment.

Li B, Vachali PP, Gorusupudi A, Shen Z, Sharifzadeh H, Besch BM, Nelson K, Horvath MM, Frederick JM, Baehr W, Bernstein PS.

Proc Natl Acad Sci U S A. 2014 Jul 15;111(28):10173-8. doi: 10.1073/pnas.1402526111. Epub 2014 Jun 30.


Centrin 2 is required for mouse olfactory ciliary trafficking and development of ependymal cilia planar polarity.

Ying G, Avasthi P, Irwin M, Gerstner CD, Frederick JM, Lucero MT, Baehr W.

J Neurosci. 2014 Apr 30;34(18):6377-88. doi: 10.1523/JNEUROSCI.0067-14.2014.


RNA interference gene therapy in dominant retinitis pigmentosa and cone-rod dystrophy mouse models caused by GCAP1 mutations.

Jiang L, Frederick JM, Baehr W.

Front Mol Neurosci. 2014 Apr 7;7:25. doi: 10.3389/fnmol.2014.00025. eCollection 2014. Review.


Unc119 gene deletion partially rescues the GRK1 transport defect of Pde6d (- /-) cones.

Zhang H, Frederick JM, Baehr W.

Adv Exp Med Biol. 2014;801:487-93. doi: 10.1007/978-1-4614-3209-8_62.


Germline deletion of Cetn1 causes infertility in male mice.

Avasthi P, Scheel JF, Ying G, Frederick JM, Baehr W, Wolfrum U.

J Cell Sci. 2013 Jul 15;126(Pt 14):3204-13. doi: 10.1242/jcs.128587. Epub 2013 May 2.


Phosducin-like protein 1 is essential for G-protein assembly and signaling in retinal rod photoreceptors.

Lai CW, Kolesnikov AV, Frederick JM, Blake DR, Jiang L, Stewart JS, Chen CK, Barrow JR, Baehr W, Kefalov VJ, Willardson BM.

J Neurosci. 2013 May 1;33(18):7941-51. doi: 10.1523/JNEUROSCI.5001-12.2013.


RNAi-mediated gene suppression in a GCAP1(L151F) cone-rod dystrophy mouse model.

Jiang L, Li TZ, Boye SE, Hauswirth WW, Frederick JM, Baehr W.

PLoS One. 2013;8(3):e57676. doi: 10.1371/journal.pone.0057676. Epub 2013 Mar 5.


Uncoordinated (UNC)119: coordinating the trafficking of myristoylated proteins.

Constantine R, Zhang H, Gerstner CD, Frederick JM, Baehr W.

Vision Res. 2012 Dec 15;75:26-32. doi: 10.1016/j.visres.2012.08.012. Epub 2012 Sep 19. Review.


The prenyl-binding protein PrBP/δ: a chaperone participating in intracellular trafficking.

Zhang H, Constantine R, Frederick JM, Baehr W.

Vision Res. 2012 Dec 15;75:19-25. doi: 10.1016/j.visres.2012.08.013. Epub 2012 Aug 29. Review.


Long-term RNA interference gene therapy in a dominant retinitis pigmentosa mouse model.

Jiang L, Zhang H, Dizhoor AM, Boye SE, Hauswirth WW, Frederick JM, Baehr W.

Proc Natl Acad Sci U S A. 2011 Nov 8;108(45):18476-81. doi: 10.1073/pnas.1112758108. Epub 2011 Oct 31.


Identification of StARD3 as a lutein-binding protein in the macula of the primate retina.

Li B, Vachali P, Frederick JM, Bernstein PS.

Biochemistry. 2011 Apr 5;50(13):2541-9. doi: 10.1021/bi101906y. Epub 2011 Mar 4.


Genome-wide association study of advanced age-related macular degeneration identifies a role of the hepatic lipase gene (LIPC).

Neale BM, Fagerness J, Reynolds R, Sobrin L, Parker M, Raychaudhuri S, Tan PL, Oh EC, Merriam JE, Souied E, Bernstein PS, Li B, Frederick JM, Zhang K, Brantley MA Jr, Lee AY, Zack DJ, Campochiaro B, Campochiaro P, Ripke S, Smith RT, Barile GR, Katsanis N, Allikmets R, Daly MJ, Seddon JM.

Proc Natl Acad Sci U S A. 2010 Apr 20;107(16):7395-400. doi: 10.1073/pnas.0912019107. Epub 2010 Apr 12.


The Leber congenital amaurosis protein, AIPL1, is needed for the viability and functioning of cone photoreceptor cells.

Kirschman LT, Kolandaivelu S, Frederick JM, Dang L, Goldberg AF, Baehr W, Ramamurthy V.

Hum Mol Genet. 2010 Mar 15;19(6):1076-87. doi: 10.1093/hmg/ddp571. Epub 2009 Dec 30.


Novel functions of photoreceptor guanylate cyclases revealed by targeted deletion.

Karan S, Frederick JM, Baehr W.

Mol Cell Biochem. 2010 Jan;334(1-2):141-55. doi: 10.1007/s11010-009-0322-z. Epub 2009 Dec 9. Review.


Trafficking of membrane proteins to cone but not rod outer segments is dependent on heterotrimeric kinesin-II.

Avasthi P, Watt CB, Williams DS, Le YZ, Li S, Chen CK, Marc RE, Frederick JM, Baehr W.

J Neurosci. 2009 Nov 11;29(45):14287-98. doi: 10.1523/JNEUROSCI.3976-09.2009.


Purification and partial characterization of a lutein-binding protein from human retina.

Bhosale P, Li B, Sharifzadeh M, Gellermann W, Frederick JM, Tsuchida K, Bernstein PS.

Biochemistry. 2009 Jun 9;48(22):4798-807. doi: 10.1021/bi9004478.


Naturally occurring animal models with outer retina phenotypes.

Baehr W, Frederick JM.

Vision Res. 2009 Nov;49(22):2636-52. doi: 10.1016/j.visres.2009.04.008. Epub 2009 Apr 16. Review.


Mutant prominin 1 found in patients with macular degeneration disrupts photoreceptor disk morphogenesis in mice.

Yang Z, Chen Y, Lillo C, Chien J, Yu Z, Michaelides M, Klein M, Howes KA, Li Y, Kaminoh Y, Chen H, Zhao C, Chen Y, Al-Sheikh YT, Karan G, Corbeil D, Escher P, Kamaya S, Li C, Johnson S, Frederick JM, Zhao Y, Wang C, Cameron DJ, Huttner WB, Schorderet DF, Munier FL, Moore AT, Birch DG, Baehr W, Hunt DM, Williams DS, Zhang K.

J Clin Invest. 2008 Aug;118(8):2908-16. doi: 10.1172/JCI35891.


Trafficking of membrane-associated proteins to cone photoreceptor outer segments requires the chromophore 11-cis-retinal.

Zhang H, Fan J, Li S, Karan S, Rohrer B, Palczewski K, Frederick JM, Crouch RK, Baehr W.

J Neurosci. 2008 Apr 9;28(15):4008-14. doi: 10.1523/JNEUROSCI.0317-08.2008. Erratum in: J Neurosci. 2008 Nov 5;28(45):11741.


Rpe65-/- and Lrat-/- mice: comparable models of leber congenital amaurosis.

Fan J, Rohrer B, Frederick JM, Baehr W, Crouch RK.

Invest Ophthalmol Vis Sci. 2008 Jun;49(6):2384-9. doi: 10.1167/iovs.08-1727. Epub 2008 Feb 22.


Involvement of guanylate cyclases in transport of photoreceptor peripheral membrane proteins.

Karan S, Frederick JM, Baehr W.

Adv Exp Med Biol. 2008;613:351-9. doi: 10.1007/978-0-387-74904-4_41. Review. No abstract available.


A model for transport of membrane-associated phototransduction polypeptides in rod and cone photoreceptor inner segments.

Karan S, Zhang H, Li S, Frederick JM, Baehr W.

Vision Res. 2008 Feb;48(3):442-52. Epub 2007 Oct 18. Review.


Deletion of PrBP/delta impedes transport of GRK1 and PDE6 catalytic subunits to photoreceptor outer segments.

Zhang H, Li S, Doan T, Rieke F, Detwiler PB, Frederick JM, Baehr W.

Proc Natl Acad Sci U S A. 2007 May 22;104(21):8857-62. Epub 2007 May 11.


The function of guanylate cyclase 1 and guanylate cyclase 2 in rod and cone photoreceptors.

Baehr W, Karan S, Maeda T, Luo DG, Li S, Bronson JD, Watt CB, Yau KW, Frederick JM, Palczewski K.

J Biol Chem. 2007 Mar 23;282(12):8837-47. Epub 2007 Jan 25.


Functional study of photoreceptor PDEdelta.

Zhang H, Frederick JM, Baehr W.

Adv Exp Med Biol. 2006;572:485-90. Review. No abstract available.


Mutant carbonic anhydrase 4 impairs pH regulation and causes retinal photoreceptor degeneration.

Yang Z, Alvarez BV, Chakarova C, Jiang L, Karan G, Frederick JM, Zhao Y, Sauvé Y, Li X, Zrenner E, Wissinger B, Hollander AI, Katz B, Baehr W, Cremers FP, Casey JR, Bhattacharya SS, Zhang K.

Hum Mol Genet. 2005 Jan 15;14(2):255-65. Epub 2004 Nov 24.


Receptor for advanced glycation end products and age-related macular degeneration.

Howes KA, Liu Y, Dunaief JL, Milam A, Frederick JM, Marks A, Baehr W.

Invest Ophthalmol Vis Sci. 2004 Oct;45(10):3713-20.


Identification and characterization of a Pi isoform of glutathione S-transferase (GSTP1) as a zeaxanthin-binding protein in the macula of the human eye.

Bhosale P, Larson AJ, Frederick JM, Southwick K, Thulin CD, Bernstein PS.

J Biol Chem. 2004 Nov 19;279(47):49447-54. Epub 2004 Sep 7.


Progressive pathways in age-related macular degeneration.

Howes KA, Frederick JM, Marks A, Baehr W.

Adv Exp Med Biol. 2003;533:91-6. Review. No abstract available.


Photoreceptor cGMP phosphodiesterase delta subunit (PDEdelta) functions as a prenyl-binding protein.

Zhang H, Liu XH, Zhang K, Chen CK, Frederick JM, Prestwich GD, Baehr W.

J Biol Chem. 2004 Jan 2;279(1):407-13. Epub 2003 Oct 15.


Retinal remodeling triggered by photoreceptor degenerations.

Jones BW, Watt CB, Frederick JM, Baehr W, Chen CK, Levine EM, Milam AH, Lavail MM, Marc RE.

J Comp Neurol. 2003 Sep 8;464(1):1-16.


Identification and light-dependent translocation of a cone-specific antigen, cone arrestin, recognized by monoclonal antibody 7G6.

Zhang H, Cuenca N, Ivanova T, Church-Kopish J, Frederick JM, MacLeish PR, Baehr W.

Invest Ophthalmol Vis Sci. 2003 Jul;44(7):2858-67.


GCAP1 rescues rod photoreceptor response in GCAP1/GCAP2 knockout mice.

Howes KA, Pennesi ME, Sokal I, Church-Kopish J, Schmidt B, Margolis D, Frederick JM, Rieke F, Palczewski K, Wu SM, Detwiler PB, Baehr W.

EMBO J. 2002 Apr 2;21(7):1545-54.


Characterization of human GRK7 as a potential cone opsin kinase.

Chen CK, Zhang K, Church-Kopish J, Huang W, Zhang H, Chen YJ, Frederick JM, Baehr W.

Mol Vis. 2001 Dec 21;7:305-13.


Mutant rhodopsin transgene expression on a null background.

Frederick JM, Krasnoperova NV, Hoffmann K, Church-Kopish J, Rüther K, Howes K, Lem J, Baehr W.

Invest Ophthalmol Vis Sci. 2001 Mar;42(3):826-33.


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