Amy Kiernan
| Title | Assistant Professor |
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| Institution | School of Medicine and Dentistry |
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| Department | Ophthalmology |
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| Address | University of Rochester Medical Center
School of Medicine and Dentistry
601 Elmwood Ave, Box 659
Rochester NY 14642
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| Title | Assistant Professor |
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| Institution | School of Medicine and Dentistry |
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| Department | Biomedical Genetics |
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Sensory organs are unique structures in the body plan of all multicellular organisms that allow perception of the environment. Our lab uses the power of mouse genetics to understand the fundamental molecular pathways involved in sensory development and disease. The eye and the inner ear of the mouse are very similar to humans and thus can serve as good models for understanding key developmental processes. As in humans, many of the specialized cell types in the ear and the eye of the mouse, such as hair cells, photoreceptors and ganglion cells, cannot regenerate when damaged due to genetics, environmental factors, or normal aging. Loss of these important cells leads to irreversible deafness, vestibular dysfunction and vision loss. Therefore, identifying the key molecules involved in the specification of these cell types and their precursors will be important when developing therapies using stem cells, progenitor cells, or gene therapy as well as in understanding the congenital defects associated with mutations in these genes. The long-term goal of this laboratory is to use advanced mouse genetic approaches, including conditional gene targeting and inducible expression systems, to elucidate key molecular pathways involved in different aspects of sensory development. Currently, our lab is studying the role of the Notch signaling pathway and the transcription factor SOX2 in the development of the eye and the inner ear. By understanding some of the basic molecular processes by which sensory organs form and are maintained, we can begin to develop therapies for prevention, repair and regeneration in sense organ disease.
Current projects:
• Dissecting the molecular pathways leading to anterior segment dysgenesis (ASD) of the eye and its contribution to developmental glaucoma.
• The role of the Notch ligand JAGGED1 during sensory precursor development in the inner ear
• The role of the transcription factor SOX2 in the development of the prosensory lineage in the inner ear
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Zhou Y, Tanzie C, Yan Z, Chen S, Duncan M, Gaudenz K, Li H, Seidel C, Lewis B, Moran A, Libby RT, Kiernan AE, Xie T. Notch2 regulates BMP signaling and epithelial morphogenesis in the ciliary body of the mouse eye. Proc Natl Acad Sci U S A. 2013 May 28; 110(22):8966-71.
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Liu K, Jiang M, Lu Y, Chen H, Sun J, Wu S, Ku WY, Nakagawa H, Kita Y, Natsugoe S, Peters JH, Rustgi A, Onaitis MW, Kiernan A, Chen X, Que J. Sox2 cooperates with inflammation-mediated stat3 activation in the malignant transformation of foregut Basal progenitor cells. Cell Stem Cell. 2013 Mar 7; 12(3):304-15.
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Steffes G, Lorente-Cánovas B, Pearson S, Brooker RH, Spiden S, Kiernan AE, Guénet JL, Steel KP. Mutanlallemand (mtl) and Belly Spot and Deafness (bsd) Are Two New Mutations of Lmx1a Causing Severe Cochlear and Vestibular Defects. PLoS One. 2012; 7(11):e51065.
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Pan W, Jin Y, Stanger B, Kiernan AE. Notch signaling is required for the generation of hair cells and supporting cells in the mammalian inner ear. Proc Natl Acad Sci U S A. 2010 Sep 7; 107(36):15798-803.
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Kiernan AE, Li R, Hawes NL, Churchill GA, Gridley T. Genetic background modifies inner ear and eye phenotypes of jag1 heterozygous mice. Genetics. 2007 Sep; 177(1):307-11.
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Kiernan AE. The paintfill method as a tool for analyzing the three-dimensional structure of the inner ear. Brain Res. 2006 May 26; 1091(1):270-6.
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Kiernan AE, Xu J, Gridley T. The Notch ligand JAG1 is required for sensory progenitor development in the mammalian inner ear. PLoS Genet. 2006 Jan; 2(1):e4.
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Kiernan AE, Cordes R, Kopan R, Gossler A, Gridley T. The Notch ligands DLL1 and JAG2 act synergistically to regulate hair cell development in the mammalian inner ear. Development. 2005 Oct; 132(19):4353-62.
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Kiernan AE, Pelling AL, Leung KK, Tang AS, Bell DM, Tease C, Lovell-Badge R, Steel KP, Cheah KS. Sox2 is required for sensory organ development in the mammalian inner ear. Nature. 2005 Apr 21; 434(7036):1031-5.
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Hulander M, Kiernan AE, Blomqvist SR, Carlsson P, Samuelsson EJ, Johansson BR, Steel KP, Enerbäck S. Lack of pendrin expression leads to deafness and expansion of the endolymphatic compartment in inner ears of Foxi1 null mutant mice. Development. 2003 May; 130(9):2013-25.
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Kiernan AE, Erven A, Voegeling S, Peters J, Nolan P, Hunter J, Bacon Y, Steel KP, Brown SD, Guénet JL. ENU mutagenesis reveals a highly mutable locus on mouse Chromosome 4 that affects ear morphogenesis. Mamm Genome. 2002 Mar; 13(3):142-8.
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Alavizadeh A, Kiernan AE, Nolan P, Lo C, Steel KP, Bucan M. The Wheels mutation in the mouse causes vascular, hindbrain, and inner ear defects. Dev Biol. 2001 Jun 1; 234(1):244-60.
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Kiernan AE, Ahituv N, Fuchs H, Balling R, Avraham KB, Steel KP, Hrabé de Angelis M. The Notch ligand Jagged1 is required for inner ear sensory development. Proc Natl Acad Sci U S A. 2001 Mar 27; 98(7):3873-8.
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Tsai H, Hardisty RE, Rhodes C, Kiernan AE, Roby P, Tymowska-Lalanne Z, Mburu P, Rastan S, Hunter AJ, Brown SD, Steel KP. The mouse slalom mutant demonstrates a role for Jagged1 in neuroepithelial patterning in the organ of Corti. Hum Mol Genet. 2001 Mar 1; 10(5):507-12.
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Brigande JV, Kiernan AE, Gao X, Iten LE, Fekete DM. Molecular genetics of pattern formation in the inner ear: do compartment boundaries play a role? Proc Natl Acad Sci U S A. 2000 Oct 24; 97(22):11700-6.
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Nolan PM, Peters J, Strivens M, Rogers D, Hagan J, Spurr N, Gray IC, Vizor L, Brooker D, Whitehill E, Washbourne R, Hough T, Greenaway S, Hewitt M, Liu X, McCormack S, Pickford K, Selley R, Wells C, Tymowska-Lalanne Z, Roby P, Glenister P, Thornton C, Thaung C, Stevenson JA, Arkell R, Mburu P, Hardisty R, Kiernan A, Erven A, Steel KP, Voegeling S, Guenet JL, Nickols C, Sadri R, Nasse M, Isaacs A, Davies K, Browne M, Fisher EM, Martin J, Rastan S, Brown SD, Hunter J. A systematic, genome-wide, phenotype-driven mutagenesis programme for gene function studies in the mouse. Nat Genet. 2000 Aug; 25(4):440-3.
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Kiernan AE, Steel KP. Mouse homologues for human deafness. Adv Otorhinolaryngol. 2000; 56:233-43.
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Kiernan AE, Zalzman M, Fuchs H, Hrabe de Angelis M, Balling R, Steel KP, Avraham KB. Tailchaser (Tlc): a new mouse mutation affecting hair bundle differentiation and hair cell survival. J Neurocytol. 1999 Oct-Nov; 28(10-11):969-85.
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Kiernan AE, Nunes F, Wu DK, Fekete DM. The expression domain of two related homeobox genes defines a compartment in the chicken inner ear that may be involved in semicircular canal formation. Dev Biol. 1997 Nov 15; 191(2):215-29.
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