Kiernan Lab
Welcome to the Kiernan Lab
Research Overview
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
Amy Kiernan, Ph.D.
Principal Investigator
Publications
View All Publications- Notch1 is required to maintain supporting cell identity and vestibular function during maturation of the mammalian balance organs.; bioRxiv : the preprint server for biology. 2024 Jun 21.
- Corrigendum: Increased central auditory gain in 5xFAD Alzheimer's disease mice as an early biomarker candidate for Alzheimer's disease diagnosis.; Frontiers in neuroscience; Vol 17, pp. 1250244. 2023 Jul 18.
- Increased central auditory gain in 5xFAD Alzheimer's disease mice as an early biomarker candidate for Alzheimer's disease diagnosis.; Frontiers in neuroscience; Vol 17, pp. 1106570. 2023 May 26.
- Deletion of Notch1 during cochlear maturation leads to rapid supporting cell death and profound deafness.; The Journal of neuroscience : the official journal of the Society for Neuroscience. 2022 Jan 23.
- Deletion of the Notch ligand Jagged1 during cochlear maturation leads to inner hair cell defects and hearing loss.; Cell death & disease; Vol 13(11), pp. 971. 2022 Jan 18.
Contact Us
Kiernan Lab
601 Elmwood Ave, Box 659
Rochester, NY 14642