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Items: 1 to 50 of 180

1.

Rescue of M-cone Function in Aged Opn1mw-/- Mice, a Model for Late-Stage Blue Cone Monochromacy.

Deng WT, Li J, Zhu P, Freedman B, Smith WC, Baehr W, Hauswirth WW.

Invest Ophthalmol Vis Sci. 2019 Aug 1;60(10):3644-3651. doi: 10.1167/iovs.19-27079.

2.

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.

3.

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.

PMID:
30647131
4.

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.

5.

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.

PMID:
30228185
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.

PMID:
29721959
7.

Publisher Correction: Gene-based Therapy in a Mouse Model of Blue Cone Monochromacy.

Zhang Y, Deng WT, Du W, Zhu P, Li J, Xu F, Sun J, Gerstner CD, Baehr W, Boye SL, Zhao C, Hauswirth WW, Pang JJ.

Sci Rep. 2018 Mar 14;8(1):4807. doi: 10.1038/s41598-018-23131-w.

8.

Human L- and M-opsins restore M-cone function in a mouse model for human blue cone monochromacy.

Deng WT, Li J, Zhu P, Chiodo VA, Smith WC, Freedman B, Baehr W, Pang J, Hauswirth WW.

Mol Vis. 2018 Jan 8;24:17-28. eCollection 2018.

9.

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.

10.

Diabetic retinopathy, an overview.

Hartnett ME, Baehr W, Le YZ.

Vision Res. 2017 Oct;139:1-6. doi: 10.1016/j.visres.2017.07.006. Epub 2017 Aug 4.

11.

Gene-based Therapy in a Mouse Model of Blue Cone Monochromacy.

Zhang Y, Deng WT, Du W, Zhu P, Li J, Xu F, Sun J, Gerstner CD, Baehr W, Boye SL, Zhao C, Hauswirth WW, Pang JJ.

Sci Rep. 2017 Jul 27;7(1):6690. doi: 10.1038/s41598-017-06982-7. Erratum in: Sci Rep. 2018 Mar 14;8(1):4807.

12.

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.

13.

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.

14.

Knockdown of unc119c results in visual impairment and early-onset retinal dystrophy in zebrafish.

Rainy N, Etzion T, Alon S, Pomeranz A, Nisgav Y, Livnat T, Bach M, Gerstner CD, Baehr W, Gothilf Y, Stiebel-Kalish H.

Biochem Biophys Res Commun. 2016 May 13;473(4):1211-1217. doi: 10.1016/j.bbrc.2016.04.041. Epub 2016 Apr 11.

PMID:
27079236
15.

Ciliopathy-associated protein CEP290 modifies the severity of retinal degeneration due to loss of RPGR.

Rao KN, Zhang W, Li L, Ronquillo C, Baehr W, Khanna H.

Hum Mol Genet. 2016 May 15;25(10):2005-2012. Epub 2016 Mar 2.

16.

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.

17.

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.

PMID:
26427472
18.

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.

19.

Domain organization and conformational plasticity of the G protein effector, PDE6.

Zhang Z, He F, Constantine R, Baker ML, Baehr W, Schmid MF, Wensel TG, Agosto MA.

J Biol Chem. 2015 Jul 10;290(28):17131-2. doi: 10.1074/jbc.A115.647636. No abstract available.

20.

cGMP/Protein Kinase G Signaling Suppresses Inositol 1,4,5-Trisphosphate Receptor Phosphorylation and Promotes Endoplasmic Reticulum Stress in Photoreceptors of Cyclic Nucleotide-gated Channel-deficient Mice.

Ma H, Butler MR, Thapa A, Belcher J, Yang F, Baehr W, Biel M, Michalakis S, Ding XQ.

J Biol Chem. 2015 Aug 21;290(34):20880-92. doi: 10.1074/jbc.M115.641159. Epub 2015 Jun 29.

21.

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.

22.

Domain organization and conformational plasticity of the G protein effector, PDE6.

Zhang Z, He F, Constantine R, Baker ML, Baehr W, Schmid MF, Wensel TG, Agosto MA.

J Biol Chem. 2015 May 15;290(20):12833-43. doi: 10.1074/jbc.M115.647636. Epub 2015 Mar 25. Erratum in: J Biol Chem. 2015 Jul 10;290(28):17131-2.

23.

Ca(2+) and Ca(2+)-interlocked membrane guanylate cyclase signal modulation of neuronal and cardiovascular signal transduction.

Sharma RK, Baehr W, Makino CL, Duda T.

Front Mol Neurosci. 2015 Mar 6;8:7. doi: 10.3389/fnmol.2015.00007. eCollection 2015. No abstract available.

24.

Retinal cone photoreceptors require phosducin-like protein 1 for G protein complex assembly and signaling.

Tracy CM, Kolesnikov AV, Blake DR, Chen CK, Baehr W, Kefalov VJ, Willardson BM.

PLoS One. 2015 Feb 6;10(2):e0117129. doi: 10.1371/journal.pone.0117129. eCollection 2015.

25.

Membrane protein transport in photoreceptors: the function of PDEδ: the Proctor lecture.

Baehr W.

Invest Ophthalmol Vis Sci. 2014 Dec 30;55(12):8653-66. doi: 10.1167/iovs.14-16066.

26.

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.

27.

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.

28.

FLT1 genetic variation predisposes to neovascular AMD in ethnically diverse populations and alters systemic FLT1 expression.

Owen LA, Morrison MA, Ahn J, Woo SJ, Sato H, Robinson R, Morgan DJ, Zacharaki F, Simeonova M, Uehara H, Chakravarthy U, Hogg RE, Ambati BK, Kotoula M, Baehr W, Haider NB, Silvestri G, Miller JW, Tsironi EE, Farrer LA, Kim IK, Park KH, DeAngelis MM.

Invest Ophthalmol Vis Sci. 2014 May 8;55(6):3543-54. doi: 10.1167/iovs.14-14047.

29.

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.

30.

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.

31.

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.

32.

Suppressing thyroid hormone signaling preserves cone photoreceptors in mouse models of retinal degeneration.

Ma H, Thapa A, Morris L, Redmond TM, Baehr W, Ding XQ.

Proc Natl Acad Sci U S A. 2014 Mar 4;111(9):3602-7. doi: 10.1073/pnas.1317041111. Epub 2014 Feb 18.

33.

A homozygous PDE6D mutation in Joubert syndrome impairs targeting of farnesylated INPP5E protein to the primary cilium.

Thomas S, Wright KJ, Le Corre S, Micalizzi A, Romani M, Abhyankar A, Saada J, Perrault I, Amiel J, Litzler J, Filhol E, Elkhartoufi N, Kwong M, Casanova JL, Boddaert N, Baehr W, Lyonnet S, Munnich A, Burglen L, Chassaing N, Encha-Ravazi F, Vekemans M, Gleeson JG, Valente EM, Jackson PK, Drummond IA, Saunier S, Attié-Bitach T.

Hum Mutat. 2014 Jan;35(1):137-46.

34.

cGMP accumulation causes photoreceptor degeneration in CNG channel deficiency: evidence of cGMP cytotoxicity independently of enhanced CNG channel function.

Xu J, Morris L, Thapa A, Ma H, Michalakis S, Biel M, Baehr W, Peshenko IV, Dizhoor AM, Ding XQ.

J Neurosci. 2013 Sep 11;33(37):14939-48. doi: 10.1523/JNEUROSCI.0909-13.2013.

35.

Photoreceptor avascular privilege is shielded by soluble VEGF receptor-1.

Luo L, Uehara H, Zhang X, Das SK, Olsen T, Holt D, Simonis JM, Jackman K, Singh N, Miya TR, Huang W, Ahmed F, Bastos-Carvalho A, Le YZ, Mamalis C, Chiodo VA, Hauswirth WW, Baffi J, Lacal PM, Orecchia A, Ferrara N, Gao G, Young-Hee K, Fu Y, Owen L, Albuquerque R, Baehr W, Thomas K, Li DY, Chalam KV, Shibuya M, Grisanti S, Wilson DJ, Ambati J, Ambati BK.

Elife. 2013 Jun 18;2:e00324. doi: 10.7554/eLife.00324.

36.

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.

37.

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.

38.

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.

39.

AAV-mediated gene therapy in the guanylate cyclase (RetGC1/RetGC2) double knockout mouse model of Leber congenital amaurosis.

Boye SL, Peshenko IV, Huang WC, Min SH, McDoom I, Kay CN, Liu X, Dyka FM, Foster TC, Umino Y, Karan S, Jacobson SG, Baehr W, Dizhoor A, Hauswirth WW, Boye SE.

Hum Gene Ther. 2013 Feb;24(2):189-202. doi: 10.1089/hum.2012.193.

40.

Retina ciliopathies: from genes to mechanisms and treatment.

Khanna H, Baehr W.

Vision Res. 2012 Dec 15;75:1. doi: 10.1016/j.visres.2012.11.007. No abstract available.

41.

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.

42.

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.

43.

Senior-Løken syndrome: a syndromic form of retinal dystrophy associated with nephronophthisis.

Ronquillo CC, Bernstein PS, Baehr W.

Vision Res. 2012 Dec 15;75:88-97. doi: 10.1016/j.visres.2012.07.003. Epub 2012 Jul 20. Review.

44.

Chemical chaperone TUDCA preserves cone photoreceptors in a mouse model of Leber congenital amaurosis.

Zhang T, Baehr W, Fu Y.

Invest Ophthalmol Vis Sci. 2012 Jun 5;53(7):3349-56. doi: 10.1167/iovs.12-9851.

45.

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.

46.

Proliferative reactive gliosis is compatible with glial metabolic support and neuronal function.

Vázquez-Chona FR, Swan A, Ferrell WD, Jiang L, Baehr W, Chien WM, Fero M, Marc RE, Levine EM.

BMC Neurosci. 2011 Oct 10;12:98. doi: 10.1186/1471-2202-12-98.

47.

Targeting of mouse guanylate cyclase 1 (Gucy2e) to Xenopus laevis rod outer segments.

Karan S, Tam BM, Moritz OL, Baehr W.

Vision Res. 2011 Nov;51(21-22):2304-11. doi: 10.1016/j.visres.2011.09.001. Epub 2011 Sep 12.

48.

Retinoid receptors trigger neuritogenesis in retinal degenerations.

Lin Y, Jones BW, Liu A, Tucker JF, Rapp K, Luo L, Baehr W, Bernstein PS, Watt CB, Yang JH, Shaw MV, Marc RE.

FASEB J. 2012 Jan;26(1):81-92. doi: 10.1096/fj.11-192914. Epub 2011 Sep 22.

49.

UNC119 is required for G protein trafficking in sensory neurons.

Zhang H, Constantine R, Vorobiev S, Chen Y, Seetharaman J, Huang YJ, Xiao R, Montelione GT, Gerstner CD, Davis MW, Inana G, Whitby FG, Jorgensen EM, Hill CP, Tong L, Baehr W.

Nat Neurosci. 2011 Jun 5;14(7):874-80. doi: 10.1038/nn.2835.

50.

Enzymatic properties and regulation of the native isozymes of retinal membrane guanylyl cyclase (RetGC) from mouse photoreceptors.

Peshenko IV, Olshevskaya EV, Savchenko AB, Karan S, Palczewski K, Baehr W, Dizhoor AM.

Biochemistry. 2011 Jun 28;50(25):5590-600. doi: 10.1021/bi200491b. Epub 2011 Jun 6.

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