Jennifer Lentz, Ph.D.
Research Assistant Professor of Otorhinolaryngology & Biocommunications
Adjunct Research Assistant Professor of Ophthalmology
Member of the Neuroscience Center of Excellence, LSUHSC
LSUHSC 2020 Gravier Street, 8th Floor
New Orleans, LA 70112
Office: (504) 599-0400
Lab: (504) 599-0892
Fax: (504) 599-0891
Usher Syndrome in Louisiana
BA, Biology and Chemistry, May 1992, Monmouth College, Monmouth, Illinois
PhD, Genetics, May, 2007, Louisiana State University Health Sciences Center, Louisiana
1992-1993 Technician, Abbott Laboratories, Abbott Park, IL
1993-1995 Research Technician, Mosier Lab, The Scripps Research Institute, San Diego, CA
1996-1997 Technician, Quidel Corporation, San Diego, CA
1997-2001 Research and Development Chemist, Prometheus Laboratories, Inc., San Diego, CA
2001-2002 Research Associate III, Elizabeth Mannick MD, Department of Pediatrics, LSUHSC
2002-2007 Research Associate III and PhD Student, Bronya Keats PhD (mentor), Department of Genetics, LSUHSC
2007-2008 Postdoctoral Researcher, Bronya Keats PhD (mentor), Department of Genetics, LSUHSC
2008-2011 Postdoctoral Researcher, Nicolas Bazan MD,PhD (mentor), Neuroscience Center, LSUHSC
2012-present Research Assistant Professor, Department of Otorhinolaryngology and Biocommunications and member of the Neuroscience Center of Excellence, LSUHSC
In The News
Rescue from Hearing Loss in Usher's Syndrome
Karen B. Avraham, Ph.D.
N Engl J Med 2013; 369:1758-1760 October 31, 2013 DOI: 10.1056/NEJMcibr1311048
Dr. Hamilton Farris, Mette Flatt, Dr. William Gordon,
Dr. Jennifer Lentz and the mouse and Dr. Nicolas Bazan (the research team)
Local medical research team makes breakthrough in curing deafness Meg Farris, WWWLTV.com, February, 4 2013.
link to page
Genetic Patch 'stops deafness' in newborn mice. J. Gallagher, BBC News Health, February 4, 2013.
Researchers Develop Rx for Deafness, Impaired Balance in Mouse Model of Usher syndrome. Provided by LSUHSC to Science Daily via EurekaAlert!, February 4, 2013.
Scientists Prevent Development of Deafness in Animals Engineered to have Usher Syndrome. Provided by RFUMC to Science Daily via EurekaAlert!, February 4, 2013.
Researchers Devolop Rx fro Deafness, Impaired Balance in Mouse Model of Usher Syndrome. Provided by LSUHSC, Science Newsline Medicine & Health Care. February 4, 2013.
Gene Therapy in mice gives hope to the deaf. A France-Presse, The Raw Story, February 4, 2013.
Partial fix for Usher syndrome found in mice. The Associated Press, USA Today, February, 9, 2013.
Molecular therapy: Deafness Diverted. Nature Publishing Group, February 13, 2013.
LSU Health Sciences Center Discovers Breakthrough Therapy to Cure Deafness. J Ballard, Silicon Bayou News, February 19, 2013
Jennifer J. Lentz Laboratory for Usher syndrome Research
The overall goal of my research is to develop a therapeutic approach to prevent or cure the deafness and blindness associated with Usher syndrome (Usher), the most common genetic cause of combined deafness and blindness. Currently, there are 3 clinical sub-types of Usher syndrome based on the severity and age of onset of deafness and blindness, and in some patients, the presence of vestibular areflexia (balance disorder). Genetically, there are 12 known genes associated with Usher syndrome; 6 for Usher type 1, 3 for Usher type 2, and 2 for Usher type 3. At LSUHSC-NO we focus on Usher syndrome type 1C, which affects the Acadian populations of south Louisiana and Canada. Approximately 6-8% of type 1 Usher cases are caused by mutations in the USH1C gene, which encodes the protein harmonin. The USH1C.216G>A (216A) mutation accounts for all cases of Usher 1 in Acadian populations. This mutation creates a cryptic splice site that is used preferentially over the authentic splice site of exon 3, which results in a frameshift that creates a stop codon in exon 4, thus producing a truncated harmonin protein. My laboratory created a model of USH1C by knocking-in the 216A mutation responsible for the combined deafness, blindness and vestibular dysfunction in an Acadian patient of south Louisiana. The Usher mutant mice also have profound congenital deafness, vestibular dysfunction and retinal degeneration characteristic of human Usher. My laboratory uses this Usher mouse model to understand the underlying mechanisms that lead to the dual sensory loss associated with Usher syndrome, and to develop therapies aimed at preventing or curing deafness and blindness.
Lentz J, Savas S, Ng SS, Athas G, Deininger P, Keats B. The USH1c 216G>A splice site mutation results in a 35-base-pair deletion. Human Genetics, 2005 116:225-227.
Lentz J, Pan F, Ng SS, Deininger P, Keats B. Ush1c216A knock-in mouse survives Katrina. Mutatation Research, 2007 616:139-144.
Lentz JJ, Gordon WC, Farris HE, MacDonald GH, Cunningham DE, Robbins CA, Tempel BL, Bazan NG, Rubel EW, Oesterle EC, Keats BJ. Deafness and retinal degeneration in a novel USH1C knock-in mouse model. Developmental Neurobiology, 2010 70:254-267.
Lentz J, Jodelka FM, Hinrick AJ, McCaffrey KE, Farris HE, Spalitta MJ, Bazan NG, Duelli DM, Rigo F, Hastings ML. Rescue of hearing and vestibular function by antisense oligonucleotides in a mouse model of human deafness. Nature Medicine, 2013 19:345-350.
Depreux FF, Wang L, Jiang H, Jodelka FM, Rosencrans RF, Rigo R, Lentz JJ, Brigande JV, Hastings, ML. Antisense Oligonucleotides delivered to the amniotic cavity in utero modulate gene expression in the postnatal mouse. Breakthrough Article: Nucleic Acids Research, 2016 Sep 26 [Epub ahead of print].
Membership in Professional Organizations
The Association for Research in Otolaryngology
The Association for Research in Vision and Ophthalmology
Society for Neuroscience
Association for Women in Science
P30 GM103340 (PI: Bazan, Pilot Project PI: Lentz)
Title: Molecular Mechanisms of USH1C disease and genetic correction in Usher mice
La CATS Center Pilot Grants Program (PI: Lentz)
Title: Molecular epidemiology and natural history of Acadian Usher syndrome
1R01DC012596-01A1 (PI: Hastings, Co-investigator: Lentz)
Title: Correction of Hearing and Vestibular Defects in a Mouse Model for Deafness
FFB Early Translational Research Award Program FY13 (PI: Lentz)
Foundation Fighting Blindness, 2013-2016
Title: An Antisense-Oligonucleotide Treatment for Retinitis Pigmentosa in Usher syndrome
Donation to LSU Foundation for Usher Syndrome Research (PI: Lentz)
Eye on Jacob Foundation and Helping Hunter, 2014
Usher 2020 Foundation, 2014 - 2017