Nrl

Summary

Gene Symbol: Nrl
Description: neural retina leucine zipper gene
Alias: D14H14S46E, neural retina-specific leucine zipper protein
Species: mouse

Top Publications

  1. ncbi Regulation of lens fiber cell differentiation by transcription factor c-Maf
    S Kawauchi
    Center for Tsukuba Advanced Research Alliance and Institute of Basic Medical Sciences, University of Tsukuba, Tsukuba 305 8577, Japan
    J Biol Chem 274:19254-60. 1999
  2. ncbi RPE65 is essential for the function of cone photoreceptors in NRL-deficient mice
    Andreas Wenzel
    Laboratory for Retinal Cell Biology, Eye Clinic, University Hospital Zurich, Zurich, Switzerland
    Invest Ophthalmol Vis Sci 48:534-42. 2007
  3. pmc MATH5 controls the acquisition of multiple retinal cell fates
    Liang Feng
    Flaum Eye Institute, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA
    Mol Brain 3:36. 2010
  4. ncbi Cone-like morphological, molecular, and electrophysiological features of the photoreceptors of the Nrl knockout mouse
    Lauren L Daniele
    F M Kirby Center for Molecular Ophthalmology, Department of Ophthalmology, School of Medicine, University of Pennsylvania, Philadelphia, 19104, USA
    Invest Ophthalmol Vis Sci 46:2156-67. 2005
  5. pmc Preservation of cone photoreceptors after a rapid yet transient degeneration and remodeling in cone-only Nrl-/- mouse retina
    Jerome E Roger
    Neurobiology Neurodegeneration and Repair Laboratory, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Neurosci 32:528-41. 2012
  6. pmc Excess cones in the retinal degeneration rd7 mouse, caused by the loss of function of orphan nuclear receptor Nr2e3, originate from early-born photoreceptor precursors
    Hong Cheng
    Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI, USA
    Hum Mol Genet 20:4102-15. 2011
  7. pmc Combinatorial regulation of photoreceptor differentiation factor, neural retina leucine zipper gene NRL, revealed by in vivo promoter analysis
    Marie Audrey I Kautzmann
    Neurobiology Neurodegeneration and Repair Laboratory, NEI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 286:28247-55. 2011
  8. pmc Differences in RDS trafficking, assembly and function in cones versus rods: insights from studies of C150S-RDS
    Dibyendu Chakraborty
    Department of Cell Biology, University of Oklahoma Health Sciences Center, 940 Stanton L Young Boulevard, BMSB 781, Oklahoma City, OK 73104, USA
    Hum Mol Genet 19:4799-812. 2010
  9. pmc Two transcription factors can direct three photoreceptor outcomes from rod precursor cells in mouse retinal development
    Lily Ng
    NIDDK, Clinical Endocrinology Branch, National Institutes of Health, Bethesda, Maryland 20892 1772, USA
    J Neurosci 31:11118-25. 2011
  10. pmc Defective photoreceptor phagocytosis in a mouse model of enhanced S-cone syndrome causes progressive retinal degeneration
    Debarshi Mustafi
    Department of Pharmacology, School of Medicine, Case Western Reserve University, Cleveland, Ohio 44106 4965, USA
    FASEB J 25:3157-76. 2011

Scientific Experts

  • Enrica Strettoi
  • Xi Qin Ding
  • STEVEN PITTLER
  • Krzysztof Palczewski
  • Joseph Corbo
  • S S Apte
  • Anand Swaroop
  • Guang Hua Peng
  • Jerome E Roger
  • Muna I Naash
  • Shiming Chen
  • Alan J Mears
  • Hong Cheng
  • Cynthia L Montana
  • Shannon M Conley
  • Jung Woong Kim
  • Cheryl M Craft
  • Saravanan Kolandaivelu
  • Artur V Cideciyan
  • Lauren L Daniele
  • Rafal Farjo
  • Kecia L Feathers
  • Xuemei Zhu
  • Edwin C T Oh
  • Edward N Pugh
  • Michael W Stuck
  • Samuel G Jacobson
  • Naoyuki Tanimoto
  • Christian Grimm
  • Mathias W Seeliger
  • Matthew Brooks
  • Andreas Wenzel
  • Jindan Yu
  • Hemant Khanna
  • Ritu Khanna
  • Ammaji Rajala
  • Chunqiao Liu
  • Masayuki Akimoto
  • Sergei S Nikonov
  • Nicholas P Boyer
  • Lucia Ziccardi
  • Emma L West
  • Anna Egger
  • Arjun Thapa
  • Rivka A Rachel
  • Kyung Hee Choi
  • Alejandro J Roman
  • Chul Hong Kim
  • Alexander Sumaroka
  • Marie Audrey I Kautzmann
  • Jimmy de Melo
  • Sang Min Jang
  • Joo Hee An
  • Jingyu Yao
  • Debarshi Mustafi
  • Lily Ng
  • William W Hauswirth
  • Yiannis Koutalos
  • Janet R Sparrow
  • Rosalie K Crouch
  • Marie Paule Felder-Schmittbuhl
  • Douglas S Kim
  • Arthur Krigel
  • Rosanne M Yetemian
  • Laszlo Hackler
  • Liang Feng
  • Connie A Myers
  • Visvanathan Ramamurthy
  • Dibyendu Chakraborty
  • Freddi I Zuniga
  • Ekaterina S Lobanova
  • Naheed W Khan
  • Kannan Kunchithapautham
  • Chunhe Chen
  • M Dominik Fischer
  • A Swaroop
  • Jared D Chrispell
  • Tomas S Aleman
  • Debra A Thompson
  • Alexander V Matveev
  • Deepak A Lamba
  • Yogita Kanan
  • Alexander B Quiambao
  • Timothy H C Hsiau
  • David Hicks
  • Mary A Raven
  • P K Swain
  • A J Mears
  • Steven J Fliesler
  • Constance L Cepko

Detail Information

Publications75

  1. ncbi Regulation of lens fiber cell differentiation by transcription factor c-Maf
    S Kawauchi
    Center for Tsukuba Advanced Research Alliance and Institute of Basic Medical Sciences, University of Tsukuba, Tsukuba 305 8577, Japan
    J Biol Chem 274:19254-60. 1999
    ..for members of the large Maf family, which are expressed in the mouse lens, and found three, c-Maf, MafB, and Nrl. Of these, the earliest factor expressed in the lens was c-Maf...
  2. ncbi RPE65 is essential for the function of cone photoreceptors in NRL-deficient mice
    Andreas Wenzel
    Laboratory for Retinal Cell Biology, Eye Clinic, University Hospital Zurich, Zurich, Switzerland
    Invest Ophthalmol Vis Sci 48:534-42. 2007
    ..In the current study, neural retina leucine zipper-deficient (Nrl(-/-)) and rod opsin (Rho(-/-))-deficient mice were used, two mouse models that have been described as having a "..
  3. pmc MATH5 controls the acquisition of multiple retinal cell fates
    Liang Feng
    Flaum Eye Institute, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA
    Mol Brain 3:36. 2010
    ..Collectively, these data suggest that Math5 regulates the generation of multiple retinal cell types via different mechanisms during retinogenesis...
  4. ncbi Cone-like morphological, molecular, and electrophysiological features of the photoreceptors of the Nrl knockout mouse
    Lauren L Daniele
    F M Kirby Center for Molecular Ophthalmology, Department of Ophthalmology, School of Medicine, University of Pennsylvania, Philadelphia, 19104, USA
    Invest Ophthalmol Vis Sci 46:2156-67. 2005
    To test the hypothesis that Nrl(-)(/)(-) photoreceptors are cones, by comparing them with WT rods and cones using morphological, molecular, histochemical, and electrophysiological criteria.
  5. pmc Preservation of cone photoreceptors after a rapid yet transient degeneration and remodeling in cone-only Nrl-/- mouse retina
    Jerome E Roger
    Neurobiology Neurodegeneration and Repair Laboratory, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Neurosci 32:528-41. 2012
    ..and homeostasis are dependent on the basic motif leucine zipper transcription factor neural retina leucine zipper (NRL)...
  6. pmc Excess cones in the retinal degeneration rd7 mouse, caused by the loss of function of orphan nuclear receptor Nr2e3, originate from early-born photoreceptor precursors
    Hong Cheng
    Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI, USA
    Hum Mol Genet 20:4102-15. 2011
    The orphan nuclear receptor NR2E3 is a direct transcriptional target of NRL, the key basic motif leucine zipper transcription factor that dictates rod versus cone photoreceptor cell fate in the mammalian retina...
  7. pmc Combinatorial regulation of photoreceptor differentiation factor, neural retina leucine zipper gene NRL, revealed by in vivo promoter analysis
    Marie Audrey I Kautzmann
    Neurobiology Neurodegeneration and Repair Laboratory, NEI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 286:28247-55. 2011
    ..The bZIP transcription factor NRL (neural retina leucine zipper) is critical for rod versus cone photoreceptor cell fate choice during retinal ..
  8. pmc Differences in RDS trafficking, assembly and function in cones versus rods: insights from studies of C150S-RDS
    Dibyendu Chakraborty
    Department of Cell Biology, University of Oklahoma Health Sciences Center, 940 Stanton L Young Boulevard, BMSB 781, Oklahoma City, OK 73104, USA
    Hum Mol Genet 19:4799-812. 2010
    ..Here, we express oligomerization-incompetent RDS (C150S-RDS) in the cone-dominant nrl(-/-) mouse...
  9. pmc Two transcription factors can direct three photoreceptor outcomes from rod precursor cells in mouse retinal development
    Lily Ng
    NIDDK, Clinical Endocrinology Branch, National Institutes of Health, Bethesda, Maryland 20892 1772, USA
    J Neurosci 31:11118-25. 2011
    ..Mice deleted for thyroid hormone receptor β2 (TRβ2) and neural retina leucine zipper factor (NRL) lack M cones and rods, respectively, but gain S cones...
  10. pmc Defective photoreceptor phagocytosis in a mouse model of enhanced S-cone syndrome causes progressive retinal degeneration
    Debarshi Mustafi
    Department of Pharmacology, School of Medicine, Case Western Reserve University, Cleveland, Ohio 44106 4965, USA
    FASEB J 25:3157-76. 2011
    ..The neural retina leucine zipper transcription factor-knockout (Nrl(-/-)) mouse model demonstrates many phenotypic features of human ESCS, including unstable S-cone-positive ..
  11. ncbi GRK1-dependent phosphorylation of S and M opsins and their binding to cone arrestin during cone phototransduction in the mouse retina
    Xuemei Zhu
    The Mary D Allen Laboratory for Vision Research, Doheny Eye Institute, Department of Cell and Neurobiology, The Keck School of Medicine of the University of Southern California, Los Angeles, California 90089 9112, USA
    J Neurosci 23:6152-60. 2003
    ..Using the "pure-cone" retinas of the neural retina leucine zipper (Nrl) knock-out (KO, -/-) mice (Mears et al...
  12. pmc Effect of Rds abundance on cone outer segment morphogenesis, photoreceptor gene expression, and outer limiting membrane integrity
    Rafal Farjo
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, USA
    J Comp Neurol 504:619-30. 2007
    ..structural, and functional consequences on cone photoreceptors of the neural retinal leucine zipper knockout (Nrl(-/-)) mice when only one allele of retinal degeneration slow (Rds) is present (Rds(+/-)/Nrl(-/-))...
  13. ncbi Nrl is required for rod photoreceptor development
    A J Mears
    Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, Michigan 48105, USA
    Nat Genet 29:447-52. 2001
    The protein neural retina leucine zipper (Nrl) is a basic motif-leucine zipper transcription factor that is preferentially expressed in rod photoreceptors. It acts synergistically with Crx to regulate rhodopsin transcription...
  14. pmc The cis-regulatory logic of the mammalian photoreceptor transcriptional network
    Timothy H C Hsiau
    Department of Pathology and Immunology, Washington University School of Medicine, St Louis, Missouri, United States of America
    PLoS ONE 2:e643. 2007
    ..and computational analysis of the network controlled by the mammalian photoreceptor transcription factors, Crx, Nrl, and Nr2e3...
  15. pmc Retention of function without normal disc morphogenesis occurs in cone but not rod photoreceptors
    Rafal Farjo
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA
    J Cell Biol 173:59-68. 2006
    ..This study provides novel insight into the distinct role of Rds in the OS development of rods and cones...
  16. pmc In vivo function of the orphan nuclear receptor NR2E3 in establishing photoreceptor identity during mammalian retinal development
    Hong Cheng
    Neuroscience Graduate Program, Department of Ophthalmology and Visual Sciences, W K Kellogg Eye Center, University of Michigan, Ann Arbor 48105, USA
    Hum Mol Genet 15:2588-602. 2006
    Rod and cone photoreceptors in mammalian retina are generated from common pool(s) of neuroepithelial progenitors. NRL, CRX and NR2E3 are key transcriptional regulators that control photoreceptor differentiation...
  17. ncbi Altered expression of genes of the Bmp/Smad and Wnt/calcium signaling pathways in the cone-only Nrl-/- mouse retina, revealed by gene profiling using custom cDNA microarrays
    Jindan Yu
    Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, Michigan 48015, USA
    J Biol Chem 279:42211-20. 2004
    ..the gene expression profile of the rod-dominated retina of wild type mouse with that of the cone-only retina of Nrl(-/-) (Neural retina leucine zipper knockout) mouse...
  18. pmc A typology of photoreceptor gene expression patterns in the mouse
    Joseph C Corbo
    Department of Pathology and Immunology, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 104:12069-74. 2007
    ..study, we carried out microarray and in situ hybridization analyses of the mouse Neural retina leucine zipper gene (Nrl) mutant, which shows an en masse conversion of rods into cones, to establish a typology of photoreceptor gene ..
  19. pmc Transformation of cone precursors to functional rod photoreceptors by bZIP transcription factor NRL
    Edwin C T Oh
    Program in Neuroscience and Department of Ophthalmology, University of Michigan, Ann Arbor, MI 48105, USA
    Proc Natl Acad Sci U S A 104:1679-84. 2007
    ..Rod photoreceptor differentiation requires the basic motif-leucine zipper (bZIP) transcription factor NRL, because loss of Nrl in mice (Nrl-/-) results in complete transformation of rods to functional cones...
  20. ncbi Multiple phosphorylated isoforms of NRL are expressed in rod photoreceptors
    P K Swain
    Department of Ophthalmology, University of Michigan, Ann Arbor, Michigan 48105, USA
    J Biol Chem 276:36824-30. 2001
    b>NRL, a bZIP transcription factor of the Maf subfamily, interacts with the homeodomain protein CRX and synergistically regulates rhodopsin expression...
  21. pmc Targeting of GFP to newborn rods by Nrl promoter and temporal expression profiling of flow-sorted photoreceptors
    Masayuki Akimoto
    Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI 48105, USA
    Proc Natl Acad Sci U S A 103:3890-5. 2006
    The Maf-family transcription factor Nrl is a key regulator of photoreceptor differentiation in mammals. Ablation of the Nrl gene in mice leads to functional cones at the expense of rods. We show that a 2...
  22. ncbi Notch 1 inhibits photoreceptor production in the developing mammalian retina
    Ashutosh P Jadhav
    Department of Genetics and Howard Hughes Medical Institute, Harvard Medical School, 77 Avenue Louis Pasteur, Boston, MA 02115, USA
    Development 133:913-23. 2006
    ..These cone enriched mutant mice should prove to be a valuable resource for the study of this relatively rare mammalian photoreceptor cell type...
  23. doi Nrl-knockout mice deficient in Rpe65 fail to synthesize 11-cis retinal and cone outer segments
    Kecia L Feathers
    Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan 48105, USA
    Invest Ophthalmol Vis Sci 49:1126-35. 2008
    ..in terms of visual cycle mechanism, the retinal phenotype resulting from Rpe65 (retinoid isomerase I) deficiency in Nrl(-)(/)(-) mice having a single class of photoreceptors resembling wild-type cones was characterized and outcomes of ..
  24. pmc Photoreceptors of Nrl -/- mice coexpress functional S- and M-cone opsins having distinct inactivation mechanisms
    Sergei S Nikonov
    F M Kirby Center for Molecular Ophthalmology, Department of Ophthalmology, School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    J Gen Physiol 125:287-304. 2005
    The retinas of mice null for the neural retina leucine zipper transcription factor (Nrl-/-) contain no rods but are populated instead with photoreceptors that on ultrastructural, histochemical, and molecular criteria appear cone like...
  25. ncbi Functional analysis of the rod photoreceptor cGMP phosphodiesterase alpha-subunit gene promoter: Nrl and Crx are required for full transcriptional activity
    Steven J Pittler
    Department of Physiological Optics, Vision Science Research Center, School of Optometry, University of Alabama at Birmingham, 924 18th Street S, Birmingham, AL 35294, USA
    J Biol Chem 279:19800-7. 2004
    ..The most active segment contained a 177-bp upstream sequence including apparent Crx and Nrl transcription factor binding sites...
  26. pmc Crx activates opsin transcription by recruiting HAT-containing co-activators and promoting histone acetylation
    Guang Hua Peng
    Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, MO 63110, USA
    Hum Mol Genet 16:2433-52. 2007
    ..of HATs, (iii) the acetylation of histone H3, then (iv) binding of other photoreceptor transcription factors (Nrl and Nr2e3) and RNA polymerase II...
  27. ncbi The photoreceptor-specific nuclear receptor Nr2e3 interacts with Crx and exerts opposing effects on the transcription of rod versus cone genes
    Guang Hua Peng
    Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, MO 63110, USA
    Hum Mol Genet 14:747-64. 2005
    ..Altogether, our findings suggest that Nr2e3 is a dual-function transcriptional regulator that acts in concert with Crx to promote and maintain the function of rod photoreceptors...
  28. pmc Endoplasmic reticulum stress-associated cone photoreceptor degeneration in cyclic nucleotide-gated channel deficiency
    Arjun Thapa
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, USA
    J Biol Chem 287:18018-29. 2012
    ..e. CNGA3(-/-)/Nrl(-/-) and CNGB3(-/-)/Nrl(-/-) mice...
  29. pmc Lipofuscin and A2E accumulate with age in the retinal pigment epithelium of Nrl-/- mice
    Nicholas P Boyer
    Department of Ophthalmology, Medical University of South Carolina, Charleston, SC, USA
    Photochem Photobiol 88:1373-7. 2012
    ..the impact of the visual pigment and photoreceptor cell type on lipofuscin accumulation, we analyzed the RPE from Nrl(-/-) mice of various ages for lipofuscin fluorescence and A2E levels...
  30. pmc Defining the integration capacity of embryonic stem cell-derived photoreceptor precursors
    Emma L West
    Department of Genetics, UCL Institute of Ophthalmology, University College London, United Kingdom
    Stem Cells 30:1424-35. 2012
    ..Rod birth peaked at day 20 of culture and expression of the early photoreceptor markers Crx and Nrl increased until day 28. Nrl levels were low in ESC-derived populations compared with developing retinae...
  31. pmc Reprogramming of adult rod photoreceptors prevents retinal degeneration
    Cynthia L Montana
    Department of Pathology and Immunology, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 110:1732-7. 2013
    ..We hypothesized that converting adult rods into cones, via knockdown of the rod photoreceptor determinant Nrl, could make the cells resistant to the effects of mutations in rod-specific genes, thereby preventing secondary ..
  32. pmc Prickle1 is expressed in distinct cell populations of the central nervous system and contributes to neuronal morphogenesis
    Chunqiao Liu
    Neurobiology Neurodegeneration and Repair Laboratory N NRL, National Eye Institute, MSC0610, 6 Center Drive, Bethesda, MD 20892, USA
    Hum Mol Genet 22:2234-46. 2013
    ..Our studies implicate Pk1 function in axonal-dendritic development associated with the maturation of CNS neurons...
  33. pmc Insulin receptor signaling in cones
    Ammaji Rajala
    Department of Ophthalmology, University of Oklahoma Health Sciences Center and Dean A McGee Eye Institute, Oklahoma City, Oklahoma 73104, USA
    J Biol Chem 288:19503-15. 2013
    ..mRNA levels of IR signaling proteins are significantly higher in cone-dominant neural retina leucine zipper (Nrl) knock-out mouse retinas compared with wild type rod-dominant retinas...
  34. pmc PGC-1α determines light damage susceptibility of the murine retina
    Anna Egger
    Biozentrum, Division of Pharmacology Neurobiology, Biozentrum, University of Basel, Basel, Switzerland
    PLoS ONE 7:e31272. 2012
    ..Together with the observed dysregulation of PGC-1α and PGC-1β in retinitis pigmentosa mouse models, these findings thus imply that PGC-1α might be an attractive target for therapeutic approaches aimed at retinal degeneration diseases...
  35. pmc Increased cone sensitivity to ABCA4 deficiency provides insight into macular vision loss in Stargardt's dystrophy
    Shannon M Conley
    University of Oklahoma Health Sciences Center, Department of Cell Biology, Oklahoma City, OK, USA
    Biochim Biophys Acta 1822:1169-79. 2012
    ..rods. Here we utilize the cone-dominant Abca4(-/-)/Nrl(-/-) double knockout mouse to study this issue...
  36. pmc Loss of retinoschisin (RS1) cell surface protein in maturing mouse rod photoreceptors elevates the luminance threshold for light-driven translocation of transducin but not arrestin
    Lucia Ziccardi
    G B Bietti Foundation, Istituto di Ricovero e Cura a Carattere Scientifico, 00198 Rome, Italy
    J Neurosci 32:13010-21. 2012
    ..Consistent with this, transcription factors CRX and NRL, which are fundamental to maturation of rod protein expression, were reduced in ROS of Rs1-KO mice at P21 but not ..
  37. pmc Defects in the outer limiting membrane are associated with rosette development in the Nrl-/- retina
    Michael W Stuck
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States of America
    PLoS ONE 7:e32484. 2012
    The neural retinal leucine zipper (Nrl) knockout mouse is a widely used model to study cone photoreceptor development, physiology, and molecular biology in the absence of rods...
  38. pmc Transcriptional activity of neural retina leucine zipper (Nrl) is regulated by c-Jun N-terminal kinase and Tip60 during retina development
    Jung Woong Kim
    Department of Life Science BK21 program, College of Natural Sciences, Chung Ang University, Seoul, South Korea
    Mol Cell Biol 32:1720-32. 2012
    Neural retina leucine zipper (Nrl), a key basic motif leucine zipper (bZIP) transcription factor, modulates rod photoreceptor differentiation by activating rod-specific target genes...
  39. ncbi Evaluation and optimization of procedures for target labeling and hybridization of cDNA microarrays
    Jindan Yu
    Departments of Ophthalmology and Visual Sciences, Biomedical Engineering, University of Michigan, Ann Arbor, MI, USA
    Mol Vis 8:130-7. 2002
    ..Standardized protocols that consistently produce low background and high intensity hybridization with small amounts of starting RNA are needed to extract differentially expressed genes from a pool of thousands of unaltered genes...
  40. pmc XIAP therapy increases survival of transplanted rod precursors in a degenerating host retina
    Jingyu Yao
    Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, Michigan, USA
    Invest Ophthalmol Vis Sci 52:1567-72. 2011
    ..To assess the survival of rod precursor cells transplanted into the Rd9 mouse, a model of X-linked retinal degeneration, and the effect of antiapoptotic therapy with X-linked inhibitor of apoptosis (XIAP) on preventing cell loss...
  41. doi Neural retina leucine-zipper regulates the expression of Ppp2r5c, the regulatory subunit of protein phosphatase 2A, in photoreceptor development
    Jung Woong Kim
    Department of Life Science BK21 program, College of Natural Sciences, Chung Ang University, Seoul, Korea
    FEBS J 277:5051-60. 2010
    ..of protein phosphatase 2A) expression was regulated by the transcription factor neural retina leucine-zipper (Nrl) through enhancement of its transcriptional activity on the Ppp2r5c promoter...
  42. pmc Circadian-clock driven cone-like photoreceptor phagocytosis in the neural retina leucine zipper gene knockout mouse
    Arthur Krigel
    Département de Neurobiologie des Rythmes, CNRS UPR 3212, Institut des Neurosciences Cellulaires et Integratives, Strasbourg, France
    Mol Vis 16:2873-81. 2010
    ..To characterize the light and circadian control of cone photoreceptor phagocytosis in mice, we capitalized on the blue cone-like phenotype of neural retina leucine zipper gene (Nrl) null mice (Nrl(-/-)).
  43. pmc Cone photoreceptors are the main targets for gene therapy of NPHP5 (IQCB1) or NPHP6 (CEP290) blindness: generation of an all-cone Nphp6 hypomorph mouse that mimics the human retinal ciliopathy
    Artur V Cideciyan
    Department of Ophthalmology, Scheie Eye Institute, University of Pennsylvania, Philadelphia, PA 19104, USA
    Hum Mol Genet 20:1411-23. 2011
    ..To produce a model of the all-cone human fovea in NPHP6-LCA, we generated rd16;Nrl-/- double-mutant mice...
  44. pmc The Spalt family transcription factor Sall3 regulates the development of cone photoreceptors and retinal horizontal interneurons
    Jimmy de Melo
    Department of Neuroscience, Johns Hopkins University School of Medicine, 733 N Broadway Avenue, Baltimore, MD 21287, USA
    Development 138:2325-36. 2011
    ....
  45. pmc Active opsin loci adopt intrachromosomal loops that depend on the photoreceptor transcription factor network
    Guang Hua Peng
    Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 108:17821-6. 2011
    ..binding by three key photoreceptor transcription factors-cone--rod homeobox (CRX), neural retina leucine zipper (NRL), and nuclear receptor subfamily 2, group E, member 3 (NR2E3)--is required for the appropriate local chromosomal ..
  46. pmc Rod phosphodiesterase-6 (PDE6) catalytic subunits restore cone function in a mouse model lacking cone PDE6 catalytic subunit
    Saravanan Kolandaivelu
    Departments of Ophthalmology and Biochemistry, Center for Neuroscience, West Virginia University, Morgantown, West Virginia 26506, USA
    J Biol Chem 286:33252-9. 2011
    ..To study this issue, we generated a cone-dominated mouse model lacking cone PDE6 (Nrl(-/-) cpfl1)...
  47. pmc Transcriptional regulation of neural retina leucine zipper (Nrl), a photoreceptor cell fate determinant
    Cynthia L Montana
    Department of Pathology and Immunology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 286:36921-31. 2011
    The transcription factor neural retina leucine zipper (Nrl) is a critical determinant of rod photoreceptor cell fate and a key regulator of rod differentiation...
  48. pmc CRX ChIP-seq reveals the cis-regulatory architecture of mouse photoreceptors
    Joseph C Corbo
    Department of Pathology and Immunology, Washington University School of Medicine, St Louis, Missouri 63110 1024, USA
    Genome Res 20:1512-25. 2010
    ..CRX ChIP-seq was also performed on Nrl(-/-) retinas, which represent an enriched source of cone photoreceptors...
  49. pmc Neovascularization, enhanced inflammatory response, and age-related cone dystrophy in the Nrl-/-Grk1-/- mouse retina
    Rosanne M Yetemian
    Doheny Eye Institute, Department of Ophthalmology, Division of Retinal Molecular Biology, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033 9224, USA
    Invest Ophthalmol Vis Sci 51:6196-206. 2010
    ..and light exposure on cone photoreceptor survival were compared between mouse retinas of neural retina leucine zipper knockout (Nrl(-/-)) mice and double-knockout mice lacking G-protein-coupled receptor kinase 1 (Nrl(-/-)Grk1(-/-)).
  50. pmc Sumoylation of bZIP transcription factor NRL modulates target gene expression during photoreceptor differentiation
    Jerome E Roger
    Neurobiology Neurodegeneration and Repair Laboratory, NEI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 285:25637-44. 2010
    ..in the mammalian retina is critically dependent on the basic motif-leucine zipper transcription factor NRL (neural retina leucine zipper)...
  51. ncbi Afferent control of horizontal cell morphology revealed by genetic respecification of rods and cones
    Mary A Raven
    Neuroscience Research Institute, University of California at Santa Barbara, Santa Barbara, California 93106, USA
    J Neurosci 27:3540-7. 2007
    ..by innervation from their respective afferents: genetic respecification of all cones to become rods, in Crxp-Nrl transgenic mice, produces an atrophic dendritic field yet leaves the axon terminal system largely intact...
  52. pmc Slowed photoresponse recovery and age-related degeneration in cones lacking G protein-coupled receptor kinase 1
    Xuemei Zhu
    Mary D Allen Laboratory for Vision Research, Doheny Eye Institute, Department of Ophthalmology and Cell and Neurobiology, Keck School of Medicine of the University of Southern California, Los Angeles, California 90033 9224, USA
    Adv Exp Med Biol 572:133-9. 2006
  53. pmc A conserved retina-specific gene encodes a basic motif/leucine zipper domain
    A Swaroop
    Department of Opthalmology, University of Michigan, Kellogg Eye Center, Ann Arbor 48105
    Proc Natl Acad Sci U S A 89:266-70. 1992
    ..The gene for AS321 is conserved during evolution and is expressed in vertebrate retina. We propose to name the gene NRL (neural retina leucine zipper.
  54. ncbi Recruitment of the rod pathway by cones in the absence of rods
    Enrica Strettoi
    Istituto di Neuroscienze del Consiglio Nazionale delle Ricerche, 56100 Pisa, Italy
    J Neurosci 24:7576-82. 2004
    In mammalian retinas, rods and cones connect to distinct sets of bipolar cells. In the retina of Nrl (neural retina leucine zipper) knock-out mice, in which rods fail to form and all photoreceptors are cones, rod bipolar cells have ..
  55. ncbi Connexin 36 in photoreceptor cells: studies on transgenic rod-less and cone-less mouse retinas
    Loan Dang
    Eye Research Institute, Oakland University, Rochester, MI 48309 4481, USA
    Mol Vis 10:323-7. 2004
    ..It remains unresolved, however, whether rods, cones or both express connexin 36. We have tried to address this question with the use of transgenic rod-less and cone-less mice...
  56. ncbi Interaction of retinal bZIP transcription factor NRL with Flt3-interacting zinc-finger protein Fiz1: possible role of Fiz1 as a transcriptional repressor
    Kenneth P Mitton
    Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI 48105, USA
    Hum Mol Genet 12:365-73. 2003
    b>NRL (neural retina leucine zipper) is a basic motif leucine zipper transcription factor of the Maf-subfamily. Multiple phosphorylated isoforms of NRL are detected specifically in rod photoreceptors...
  57. ncbi Temporal and spatial expression patterns of the CRX transcription factor and its downstream targets. Critical differences during human and mouse eye development
    L C Bibb
    Section of Cell and Molecular Biology, Imperial College School of Medicine, Sir Alexander Fleming Building, Exhibition Road, London SW7 2AZ, UK
    Hum Mol Genet 10:1571-9. 2001
    ..These data support concerns about the extent to which we can extrapolate from rodent models regarding embryonic development and disease pathophysiology...
  58. ncbi The matrix metalloproteinase-14 (MMP-14) gene is structurally distinct from other MMP genes and is co-expressed with the TIMP-2 gene during mouse embryogenesis
    S S Apte
    Department of Biomedical Engineering, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    J Biol Chem 272:25511-7. 1997
    ..The stringent co-expression of these two genes suggests common regulatory pathways that may have important functional implications for the activation of pro-gelatinase A in health and disease...
  59. ncbi Expression of the bZIP transcription factor gene Nrl in the developing nervous system
    Q Liu
    Division of Molecular and Developmental Biology, Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada
    Oncogene 12:207-11. 1996
    Proteins of the Maf/Nrl subfamily of bZIP transcription factors are involved in the regulation of tissue-specific gene expression...
  60. ncbi Molecular characterization of the murine neural retina leucine zipper gene, Nrl
    Q Farjo
    Department of Ophthalmology, Kellogg Eye Center, University of Michigan, Ann Arbor 48105
    Genomics 18:216-22. 1993
    The NRL gene (D14S46E) is expressed in cells of human retina and encodes a putative DNA-binding protein of the leucine zipper family. Here we describe the analysis of the murine homolog of the NRL gene, Nrl...
  61. pmc Novel distribution of junctional adhesion molecule-C in the neural retina and retinal pigment epithelium
    Lauren L Daniele
    F M Kirby Center for Molecular Ophthalmology, Department of Ophthalmology, School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Comp Neurol 505:166-76. 2007
    ..Recent studies reported that Jam-C mRNA was increased threefold in the all-cone retina of the Nrl(-/-) mouse, suggesting that Jam-C is required for maturation and polarization of cone photoreceptors cells...
  62. pmc Retinoid processing in cone and Müller cell lines
    Yogita Kanan
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA
    Exp Eye Res 86:344-54. 2008
    ..Alternatively, this could be due to the potential that rMC-1, as a transformed cell line, has stopped expressing the proteins needed for the regeneration of 11-cis retinoids...
  63. doi Functional activity of photoreceptor cyclic nucleotide-gated channels is dependent on the integrity of cholesterol- and sphingolipid-enriched membrane domains
    Xi Qin Ding
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Dean A McGee Eye Institute, Oklahoma City, Oklahoma 73104, USA
    Biochemistry 47:3677-87. 2008
    ....
  64. pmc Mechanistic basis for the failure of cone transducin to translocate: why cones are never blinded by light
    Ekaterina S Lobanova
    Albert Eye Research Institute, Duke University, Durham, North Carolina 27710, USA
    J Neurosci 30:6815-24. 2010
    ....
  65. pmc Deciphering the structure and function of Als2cr4 in the mouse retina
    Freddi I Zuniga
    Mary D Allen Laboratory for Vision Research, Doheny Eye Institute, Los Angeles, CA 90033 9224, USA
    Invest Ophthalmol Vis Sci 51:4407-15. 2010
    ..Previous in situ hybridization and gene expression profiles show that the mRNAs encoding Als2cr4 are abundant in the eye, hippocampus, cerebellum, and olfactory bulb...
  66. pmc MicroRNA profile of the developing mouse retina
    Laszlo Hackler
    Departments of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Invest Ophthalmol Vis Sci 51:1823-31. 2010
    ..Conclusions. Global expression profiling revealed dozens of miRNAs with significant expression changes in the developing retina. Precise patterns of expression of miRNAs suggest their specific roles in development...
  67. pmc Noninvasive, in vivo assessment of mouse retinal structure using optical coherence tomography
    M Dominik Fischer
    Division of Ocular Neurodegeneration, Centre for Ophthalmology, Institute for Ophthalmic Research, University of Tuebingen, Tuebingen, Germany
    PLoS ONE 4:e7507. 2009
    ..Optical coherence tomography (OCT) is a novel method of retinal in vivo imaging. In this study, we assessed the potential of OCT to yield histology-analogue sections in mouse models of retinal degeneration...
  68. pmc Impaired cone function and cone degeneration resulting from CNGB3 deficiency: down-regulation of CNGA3 biosynthesis as a potential mechanism
    Xi Qin Ding
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA
    Hum Mol Genet 18:4770-80. 2009
    ..Hence, we show that loss of CNGB3 reduces biosynthesis of CNGA3 and impairs cone CNG channel function. We suggest that down-regulation of CNGA3 contributes to the pathogenic mechanism by which CNGB3 mutations lead to human cone disease...
  69. pmc AIPL1, a protein associated with childhood blindness, interacts with alpha-subunit of rod phosphodiesterase (PDE6) and is essential for its proper assembly
    Saravanan Kolandaivelu
    Department of Ophthalmology, Sensory Neuroscience Research Center, West Virginia University, Morgantown, West Virginia 26506, USA
    J Biol Chem 284:30853-61. 2009
    ..Our studies establish that AIPL1 interacts with the catalytic subunit (alpha) of PDE6 and is needed for the proper assembly of functional rod PDE6 subunits...
  70. pmc Rdh12 activity and effects on retinoid processing in the murine retina
    Jared D Chrispell
    Departments of Biological Chemistry, University of Michigan Medical School, Ann Arbor, Michigan 48105, USA
    J Biol Chem 284:21468-77. 2009
    ..were greater than in Rpe65-Met(450) mice and increased during postnatal development in wild-type mice and Nrl-deficient mice having an all-cone retina...
  71. doi Cone outer segment morphology and cone function in the Rpe65-/- Nrl-/- mouse retina are amenable to retinoid replacement
    Kannan Kunchithapautham
    Department of Neurosciences, Division of Research, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Invest Ophthalmol Vis Sci 50:4858-64. 2009
    ..Here, we determine the consequences of 11-cis retinal withdrawal and supplementation on cone development in the all-cone Nrl(-/-) retina.
  72. pmc Formation of all-trans retinol after visual pigment bleaching in mouse photoreceptors
    Chunhe Chen
    Department of Ophthalmology, Medical University of South Carolina, Charleston, SC 29425, USA
    Invest Ophthalmol Vis Sci 50:3589-95. 2009
    ..all-trans retinol is formed after visual pigment bleaching through the reduction of all-trans retinal in a reaction involving NADPH. This reduction begins the recycling of the chromophore for the regeneration of the visual pigment...
  73. pmc Baf60c is a component of the neural progenitor-specific BAF complex in developing retina
    Deepak A Lamba
    Department of Biological Structure, University of Washington, Seattle, Washington 98195, USA
    Dev Dyn 237:3016-23. 2008
    ..Finally, we show that Baf60c is re-expressed in the Müller glial cells that re-enter the cell cycle after neurotoxic damage...
  74. pmc Native cone photoreceptor cyclic nucleotide-gated channel is a heterotetrameric complex comprising both CNGA3 and CNGB3: a study using the cone-dominant retina of Nrl-/- mice
    Alexander V Matveev
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, USA
    J Neurochem 106:2042-55. 2008
    ..using the cone-dominant retina in mice deficient in the transcription factor neural retina leucine zipper (Nrl)...
  75. ncbi Mapping of the neural retina leucine zipper gene, Nrl, to mouse chromosome 14
    I N Bespalova
    Mental Health Research Institute, University of Michigan, Ann Arbor 48109
    Mamm Genome 4:618-20. 1993

Research Grants64

  1. INNER RETINAL NEURONS IN NORMAL AND DEGENERATING MICE
    Enrica Strettoi; Fiscal Year: 2005
    ..If selective alterations are identified, they have to be taken into account when devising new therapeutic strategies. They would also become candidates as contributors to the secondary degeneration of cones that follows loss of rods. ..
  2. Mechanism of Cone Degeneration Resulting from CNG Channel Deficiency
    Xi Qin Ding; Fiscal Year: 2010
    ..We will perform the proposed studies using the CNGA3-/-/Nrl-/- and CNGB3-/-/Nrl-/- mouse lines...
  3. STRUCTURAL STUDIES OF G PROTEIN-COUPLED RECEPTORS
    KRZYSZTOF NMI PALCZEWSKI; Fiscal Year: 2010
    ..The importance of the protein family for human health makes this effort worthwhile. ..
  4. Using Nanotechnology to Rescue Visual Loss in A Model of Human Blinding Disorder
    Xi Qin Ding; Fiscal Year: 2007
    ..Information obtained from the proposed study will open a new pathway for therapeutic interventions of retinal degeneration and will have significant impact on the clinical treatment of human retinal diseases. ..
  5. Gene Regulatory Networks in Rod Photoreceptor Cells
    Joseph Corbo; Fiscal Year: 2007
    ..In addition, a second TF required for normal rod differentiation, NRL, has been implicated in retinitis pigmentosa. The target genes of this latter TF largely remain to be identified...
  6. IMAGING OF ROD OUTER SEGMENT BY CRYO-ELECTRON TOMOGRAPHY
    Krzysztof Palczewski; Fiscal Year: 2007
    ..These studies will add significantly to our understanding of the normal and disease stage organization of the rod outer segments. ..
  7. VERTEBRATE CAROTENOID ISOMERASES
    Krzysztof Palczewski; Fiscal Year: 2005
    ..Lastly, the contribution of these enzymes to retinoid processing and, hence, to vision will be studied using transgenic animal models. ..
  8. STRUCTURAL STUDIES OF G PROTEIN-COUPLED RECEPTORS
    Krzysztof Palczewski; Fiscal Year: 2007
    ..The importance of the protein family for human health makes this effort worthwhile. ..
  9. INNER RETINAL NEURONS IN NORMAL AND DEGENERATING MICE
    Enrica Strettoi; Fiscal Year: 2007
    ..This is very important to design mutation-independent therapies. ..
  10. QUANTITATIVE ANALYSIS AND ENGINEERING OF THE PHOTORECEPTOR TRANSCRIPTION NETWORK
    Joseph C Corbo; Fiscal Year: 2010
    ..model of the photoreceptor transcriptional network controlled by the transcription factors (TFs), Crx and Nrl. In the course of this work, we developed a computational algorithm, Phastfind, to predict CREs around hundreds of ..
  11. STRUCTURAL STUDIES OF G PROTEIN-COUPLED RECEPTORS
    KRZYSZTOF NMI PALCZEWSKI; Fiscal Year: 2010
    ..The importance of the protein family for human health makes this effort worthwhile. ..
  12. Analysis of retina rod photoreceptor GARP and cGMP-gated cation channel
    Steven J Pittler; Fiscal Year: 2010
    ..The studies may also yield new targets for intervention as possible treatments for certain forms of hereditary retina degeneration. ..
  13. QUANTITATIVE ANALYSIS AND ENGINEERING OF THE PHOTORECEPTOR TRANSCRIPTION NETWORK
    Joseph Corbo; Fiscal Year: 2009
    ..model of the photoreceptor transcriptional network controlled by the transcription factors (TFs), Crx and Nrl. In the course of this work, we developed a computational algorithm, Phastfind, to predict CREs around hundreds of ..
  14. RETINOIDS IN VISION
    Krzysztof Palczewski; Fiscal Year: 2009
    ..Studies proposed in this application will add new biochemical and physiological information to steps in the retinoid cycle that are as yet poorly understood but which are critical for the normal function of photoreceptors. ..
  15. INNER RETINAL NEURONS IN NORMAL AND DEGENERATING
    Enrica Strettoi; Fiscal Year: 2009
    ..This is very important to design mutation-independent therapies. ..
  16. Analysis of retina rod photoreceptor GARP and cGMP-gated cation channel
    STEVEN PITTLER; Fiscal Year: 2009
    ..The studies may also yield new targets for intervention as possible treatments for certain forms of hereditary retina degeneration. ..
  17. RETINOIDS IN VISION
    Krzysztof Palczewski; Fiscal Year: 2007
    ..abstract_text> ..
  18. STRUCTURAL STUDIES OF ARRESTIN AND RHODOPSIN PHOSPHATASE
    Krzysztof Palczewski; Fiscal Year: 1993
    ..4) to characterize beta-arrestin, a homolog of arrestin, including its functional and immunological properties. (5) to definitively identify the rhodopsin phosphatase and an inhibitor of its activity...
  19. PHOTOTRANSDUCTION ENZYMES
    Krzysztof Palczewski; Fiscal Year: 2003
    ....
  20. STRUCTURE AND REGULATION OF RETINAL CGMP PDE ALPHA GENE
    STEVEN PITTLER; Fiscal Year: 2002
    ..Ultimately, experiments will be carried out to examine the functional consequences of PDE6 mutations that have been linked to human RP. ..
  21. STRUCTURAL STUDIES OF ARRESTINS
    Krzysztof Palczewski; Fiscal Year: 2001
    ....