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Amy Kiernan, Ph.D.

Amy Kiernan, Ph.D.

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Office (585) 273-5794

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About Me

Faculty Appointments

Associate Professor - Department of Ophthalmology (SMD)

Associate Professor - Department of Biomedical Genetics (SMD) - Joint

Credentials

Education

PhD | Boston College. Biology. 1997

BA | Coll of The Holy Cross. Biology. 1992

Research

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 mou...
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

Publications

Journal Articles

is required to maintain supporting cell identity and vestibular function during maturation of the mammalian balance organs.

Heffer A, Lee C, Holt JC, Kiernan AE

bioRxiv : the preprint server for biology.. 2024 June 21 Epub 06/21/2024.

Deletion of during Cochlear Maturation Leads to Rapid Supporting Cell Death and Profound Deafness.

Heffer A, Gilels FA, Kiernan AE

The Journal of neuroscience : the official journal of the Society for Neuroscience.. 2023 January 1143 (2):199-210. Epub 11/23/2022.

Increased central auditory gain in 5xFAD Alzheimer's disease mice as an early biomarker candidate for Alzheimer's disease diagnosis.

Na D, Zhang J, Beaulac HJ, Piekna-Przybylska D, Nicklas PR, Kiernan AE, White PM

Frontiers in neuroscience.. 2023 17 :1106570. Epub 05/26/2023.

Corrigendum: Increased central auditory gain in 5xFAD Alzheimer's disease mice as an early biomarker candidate for Alzheimer's disease diagnosis.

Na D, Zhang J, Beaulac HJ, Piekna-Przybylska D, Nicklas PR, Kiernan AE, White PM

Frontiers in neuroscience.. 2023 17 :1250244. Epub 07/18/2023.

Deletion of the Notch ligand Jagged1 during cochlear maturation leads to inner hair cell defects and hearing loss.

Gilels FA, Wang J, Bullen A, White PM, Kiernan AE

Cell death & disease.. 2022 November 1813 (11):971. Epub 11/18/2022.

Notch-mediated lateral induction is necessary to maintain vestibular prosensory identity during inner ear development.

Brown RM, Nelson JC, Zhang H, Kiernan AE, Groves AK

Developmental biology.. 2020 June 1462 (1):74-84. Epub 03/05/2020.

SOX2 is required for inner ear growth and cochlear nonsensory formation before sensory development.

Steevens AR, Glatzer JC, Kellogg CC, Low WC, Santi PA, Kiernan AE

Development.. 2019 June 21146 (13)Epub 06/21/2019.

Trabecular meshwork morphogenesis: A comparative analysis of wildtype and anterior segment dysgenesis mouse models.

Rausch RL, Libby RT, Kiernan AE

Experimental eye research.. 2018 May 170 :81-91. Epub 02/13/2018.

Deletion of a Long-Range Enhancer Disrupts Inner Ear Development in Mice.

Johnson KR, Gagnon LH, Tian C, Longo-Guess CM, Low BE, Wiles MV, Kiernan AE

Genetics.. 2018 March 208 (3):1165-1179. Epub 01/03/2018.

Ciliary margin-derived BMP4 does not have a major role in ocular development.

Rausch RL, Libby RT, Kiernan AE

PloS one.. 2018 13 (5):e0197048. Epub 05/08/2018.

SOX2 is required for inner ear neurogenesis.

Steevens AR, Sookiasian DL, Glatzer JC, Kiernan AE

Scientific reports.. 2017 June 227 (1):4086. Epub 06/22/2017.

Using genetic mouse models to gain insight into glaucoma: Past results and future possibilities.

Fernandes KA, Harder JM, Williams PA, Rausch RL, Kiernan AE, Nair KS, Anderson MG, John SW, Howell GR, Libby RT

Experimental eye research.. 2015 December 141 :42-56. Epub 06/24/2015.

LMO4 functions as a negative regulator of sensory organ formation in the mammalian cochlea.

Deng M, Luo XJ, Pan L, Yang H, Xie X, Liang G, Huang L, Hu F, Kiernan AE, Gan L

The Journal of neuroscience : the official journal of the Society for Neuroscience.. 2014 July 2334 (30):10072-7. Epub 1900 01 01.

Activated notch causes deafness by promoting a supporting cell phenotype in developing auditory hair cells.

Savoy-Burke G, Gilels FA, Pan W, Pratt D, Que J, Gan L, White PM, Kiernan AE

PloS one.. 2014 9 (9):e108160. Epub 09/29/2014.

Ectopic expression of activated notch or SOX2 reveals similar and unique roles in the development of the sensory cell progenitors in the mammalian inner ear.

Pan W, Jin Y, Chen J, Rottier RJ, Steel KP, Kiernan AE

The Journal of neuroscience : the official journal of the Society for Neuroscience.. 2013 October 933 (41):16146-57. Epub 1900 01 01.

Notch2 regulates BMP signaling and epithelial morphogenesis in the ciliary body of the mouse eye.

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

Proceedings of the National Academy of Sciences of the United States of America.. 2013 May 28110 (22):8966-71. Epub 05/15/2013.

Notch signaling during cell fate determination in the inner ear.

Kiernan AE

Seminars in cell & developmental biology.. 2013 May 24 (5):470-9. Epub 04/08/2013.

Mutanlallemand (mtl) and Belly Spot and Deafness (bsd) are two new mutations of Lmx1a causing severe cochlear and vestibular defects.

Steffes G, Lorente-Cánovas B, Pearson S, Brooker RH, Spiden S, Kiernan AE, Guénet JL, Steel KP

PloS one.. 2012 7 (11):e51065. Epub 11/30/2012.

Notch signaling is required for the generation of hair cells and supporting cells in the mammalian inner ear.

Pan W, Jin Y, Stanger B, Kiernan AE

Proceedings of the National Academy of Sciences of the United States of America.. 2010 September 7107 (36):15798-803. Epub 08/23/2010.

Genetic background modifies inner ear and eye phenotypes of jag1 heterozygous mice.

Kiernan AE, Li R, Hawes NL, Churchill GA, Gridley T

Genetics.. 2007 September 177 (1):307-11. Epub 1900 01 01.

The paintfill method as a tool for analyzing the three-dimensional structure of the inner ear.

Kiernan AE

Brain research.. 2006 May 261091 (1):270-6. Epub 04/05/2006.

The Notch ligand JAG1 is required for sensory progenitor development in the mammalian inner ear.

Kiernan AE, Xu J, Gridley T

PLoS genetics.. 2006 January 2 (1):e4. Epub 01/13/2006.

The Notch ligands DLL1 and JAG2 act synergistically to regulate hair cell development in the mammalian inner ear.

Kiernan AE, Cordes R, Kopan R, Gossler A, Gridley T

Development.. 2005 October 132 (19):4353-62. Epub 09/01/2005.

Sox2 is required for sensory organ development in the mammalian inner ear.

Kiernan AE, Pelling AL, Leung KK, Tang AS, Bell DM, Tease C, Lovell-Badge R, Steel KP, Cheah KS

Nature.. 2005 April 21434 (7036):1031-5. Epub 1900 01 01.

Lack of pendrin expression leads to deafness and expansion of the endolymphatic compartment in inner ears of Foxi1 null mutant mice.

Hulander M, Kiernan AE, Blomqvist SR, Carlsson P, Samuelsson EJ, Johansson BR, Steel KP, Enerbäck S

Development.. 2003 May 130 (9):2013-25. Epub 1900 01 01.

ENU mutagenesis reveals a highly mutable locus on mouse Chromosome 4 that affects ear morphogenesis.

Kiernan AE, Erven A, Voegeling S, Peters J, Nolan P, Hunter J, Bacon Y, Steel KP, Brown SD, Guénet JL

Mammalian genome : official journal of the International Mammalian Genome Society.. 2002 March 13 (3):142-8. Epub 1900 01 01.

The Wheels mutation in the mouse causes vascular, hindbrain, and inner ear defects.

Alavizadeh A, Kiernan AE, Nolan P, Lo C, Steel KP, Bucan M

Developmental biology.. 2001 June 1234 (1):244-60. Epub 1900 01 01.

The Notch ligand Jagged1 is required for inner ear sensory development.

Kiernan AE, Ahituv N, Fuchs H, Balling R, Avraham KB, Steel KP, Hrabé de Angelis M

Proceedings of the National Academy of Sciences of the United States of America.. 2001 March 2798 (7):3873-8. Epub 03/20/2001.

The mouse slalom mutant demonstrates a role for Jagged1 in neuroepithelial patterning in the organ of Corti.

Tsai H, Hardisty RE, Rhodes C, Kiernan AE, Roby P, Tymowska-Lalanne Z, Mburu P, Rastan S, Hunter AJ, Brown SD, Steel KP

Human molecular genetics.. 2001 March 110 (5):507-12. Epub 1900 01 01.

Molecular genetics of pattern formation in the inner ear: do compartment boundaries play a role?

Brigande JV, Kiernan AE, Gao X, Iten LE, Fekete DM

Proceedings of the National Academy of Sciences of the United States of America.. 2000 October 2497 (22):11700-6. Epub 1900 01 01.

Mouse homologues for human deafness.

Kiernan AE, Steel KP

Advances in oto-rhino-laryngology. 2000 56 :233-43. Epub 1900 01 01.

Tailchaser (Tlc): a new mouse mutation affecting hair bundle differentiation and hair cell survival.

Kiernan AE, Zalzman M, Fuchs H, Hrabe de Angelis M, Balling R, Steel KP, Avraham KB

Journal of neurocytology.. 1999 28 (10-11):969-85. Epub 1900 01 01.

The expression domain of two related homeobox genes defines a compartment in the chicken inner ear that may be involved in semicircular canal formation.

Kiernan AE, Nunes F, Wu DK, Fekete DM

Developmental biology.. 1997 November 15191 (2):215-29. Epub 1900 01 01.

In vivo gene transfer into the embryonic inner ear using retroviral vectors.

Kiernan AE, Fekete DM

Audiology & neuro-otology.. 1997 2 (1-2):12-24. Epub 1900 01 01.