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Angelika Doetzlhofer

Angelika Doetzlhofer, PhD

Highlights

Languages

  • English

Gender

Female

Johns Hopkins Affiliations:

  • Johns Hopkins School of Medicine Faculty

About Angelika Doetzlhofer

Primary Academic Title

Professor of Neuroscience

Background

Dr. Angelika Doetzlhofer is a professor of neuroscience and otolaryngology at the Johns Hopkins University School of Medicine and is affiliated with the Johns Hopkins Center for Hearing and Balance.

Dr. Doetzlhofer’s research focuses on identifying and characterizing the molecular and cellular mechanisms that regulate the formation of the mammalian auditory sensory organ and restrict its regenerative capacity. The auditory sensory organ, located in the inner ear, is essential for sound detection. Loss or dysfunction of its mechano-sensory hair cells is a primary cause of hearing loss and deafness in humans. Unlike mammals, non-mammalian vertebrates retain the ability to regenerate hair cells throughout life via supporting cells. Previous studies, including those by Dr. Doetzlhofer’s group and others, have shown that immature supporting cells in mice can regenerate hair cells in response to trauma or specific signals that induce hair cell formation. This regenerative capacity, however, diminishes as supporting cells mature. Current research objectives include: (1) reprogramming mature supporting cells into progenitor-like cells to facilitate functional hair cell formation and restore hearing in deafened mice; (2) elucidating the signals and transcription factors that govern the differentiation and maturation of supporting cells and hair cells; and (3) determining how developmental gradients influence the differentiation and tonotopic specialization of the auditory sensory organ.

This research has significant clinical potential to advance the treatment of patients with hearing and balance disorders.

She earned M.S. and Ph.D. degrees in biochemistry from the Universität Wien in Vienna, Austria.

Dr. Doetzlhofer completed a post-doctoral fellowship in molecular and developmental biology—and served as a senior research associate—at House Research Institute before joining the Johns Hopkins faculty in November 2008.

Additional Academic Titles

Professor of Otolaryngology-Head and Neck Surgery

Contact for Research Inquiries

855 North Wolfe Street
The Solomon H. Snyder Department of Neuroscience
Baltimore, MD 21205

Phone: (410) 614-9215
Fax: (443) 287-7672
adoetzlhofer@jhmi.edu

Research Interests

Auditory development, Hair cell regeneration, Hearing

Lab Website

Doetzlhofer Laboratory - Center for Sensory Biology - Lab Website

  • Auditory hair cells, located in the inner ear cochlea, are critical for our ability to detect sound. Research in Dr. Doetzlhofer's laboratory focuses on ways to identify and characterize the molecular mechanisms of hair cell development in the mammalian auditory system. She is also seeking to identify the molecular roadblocks preventing mammalian hair cell regeneration.

Research Summary

The Doetzlhofer Lab seeks to identify and characterize the gene regulatory networks that govern auditory sensory development and regeneration. The inner ear auditory sensory epithelium is critical for our ability to detect sound. Damage or loss to its mechano-sensory hair cells is permanent, leading to hearing deficits and deafness. However, in non-mammalian vertebrates, surrounding supporting cells undergo a process of de-differentiation after hair cell loss, and replace lost hair cells by either cell division or direct trans-differentiation.

Current research topics include:

  • The role of RNA binding proteins LIN28B and TRIM71 in regulating auditory progenitor behavior and supporting cell plasticity.
  • The function of Notch signaling pathway in supporting cell development and hair cell regeneration. 
  • The role of morphogen gradients in the differentiation and tonotopic specialization of the auditory organ.

Selected Publications

  • Campbell DP, Chrysostomou E, Doetzlhofer A. Canonical Notch signaling plays an instructive role in auditory supporting cell development. Sci Rep. 2016 Jan 20;6:19484. doi: 10.1038/srep19484. PMID: 26786414

  • Chrysostomou E, Zhou L, Darcy YL, Graves KA, Doetzlhofer A, Cox BC. The Notch Ligand Jagged1 Is Required for the Formation, Maintenance, and Survival of Hensen's Cells in the Mouse Cochlea. J Neurosci. 2020 Dec 2;40(49):9401-9413. doi: 10.1523/JNEUROSCI.1192-20.2020. Epub 2020 Oct 30. PMID: 33127852

  • Golden EJ, Benito-Gonzalez A, Doetzlhofer A. The RNA-binding protein LIN28B regulates developmental timing in the mammalian cochlea. Proc Natl Acad Sci U S A. 2015 Jul 21;112(29):E3864-73. doi: 10.1073/pnas.1501077112. Epub 2015 Jul 2. PMID: 26139524

  • Li XJ, Doetzlhofer A. LIN28B/let-7 control the ability of neonatal murine auditory supporting cells to generate hair cells through mTOR signaling. Proc Natl Acad Sci U S A. 2020 Sep 8;117(36):22225-22236. doi: 10.1073/pnas.2000417117. Epub 2020 Aug 21. PMID: 32826333

  • Prajapati-DiNubila M, Benito-Gonzalez A, Golden EJ, Zhang S, Doetzlhofer A. A counter gradient of Activin A and follistatin instructs the timing of hair cell differentiation in the murine cochlea. Elife. 2019 Jun 12;8:e47613. doi: 10.7554/eLife.47613. PMID: 31187730

Honors

  • Hamilton Smith Award for Innovative Research, Johns Hopkins University, 5/17/21
  • Basil O'Connor Scholar, March of Dimes Birth Defects Foundation, 2/1/11
  • Whitehall Award, Whitehall Foundation, 6/1/10

Graduate Program Affiliations

  • Neuroscience Graduate Program

    BCMB Graduate Program

Memberships

  • Society for Neuroscience,

    Member

  • Association for Research in Otolaryngology,

    Member

Additional Training

Postdoctoral fellow at House Research Institute, Los Angeles, CA (2007)

Expertise

Education

  • University of Vienna, Ph.D., 2000