Jennifer J. Lentz, Ph.D.
Associate Professor of Otorhinolaryngology &
BA, Biology and Chemistry, May 1992, Monmouth College, Monmouth, Illinois
For a full list of publications, click here
In The News
Cajun Strong, Community and Science Bolter lives of Deaf-Blind in Acadiana. Phyllis Boudoin Griffard. Louisiana Cultural Vistas, June 2017.
Hearing in the mouse of Usher. JV Brigande. Nature Biotechnology News and Views, February, 2017, 35(3):216-218.
Rescue from Hearing Loss in Usher's Syndrome, Karen B. Avraham, Ph.D. New England Journal of Medicine, 2013; 369:1758-1760 October 31, 2013 DOI: 10.1056/NEJMcibr1311048
Dr. Hamilton Farris, Mette Flatt, Dr. William Gordon,
Local medical research team makes breakthrough in curing deafness.Meg Farris, WWWLTV.com, February, 4 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 2.5% of Usher cases are caused by mutations in the USH1C gene, which encodes the protein harmonin. The USH1C.216G>A (216A) mutation accounts for nearly 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.
Ponnath A, Depreux F, Jodelka FM, Farris HE, Rigo F, Hastings ML, Lentz JJ. Rescue of outer hair cells with antisense oligonucleotides in Usher mice is dependent on age of treatment. Journal of the Association for Research in Otolaryngology, 2017, 10.1007/s 101620017-0640-x. PMID: 29027038
S Vijayakumar, F Depreux, FM Jodelka, JJ Lentz, F Rigo, TA Jones, ML Hastings. Rescue of peripheral vestibular function in Usher syndrome mice using a splice-switching antisense oligonucleotide. Human Molecular Genetics, 2017 doi.or/10.1093/hmg/ddx234. PMID: 28633508
Pan B, Askew C, Galvin A, Haman-Ackah S, Asai Y, Indzhykulian AA, Jodelka F, Hastings ML, Lentz JJ, Vandenberghe LH, Holt JR, Geleoc GGS. Gene therapy restores auditory and vestibular function in a mouse model of Usher syndrome type IC. Nature Biotechnology, 2017 35(3):264-274. PMID: 28165476
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 44(20):9519-9529. PMID: 27683224
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. PMID: 23380860
Membership in Professional Organizations
Donation to LSU Foundation for Usher Syndrome Research (PI: Lentz)
Eye on Jacob Foundation and Helping Hunter, 2014
Ush One See Foundation, 2017