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Student Seminars

Upcoming20232022202120202019

Age-related Neuropathological and Neurophysiological Changes of Auditory Processing in a Knockout Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis

Yanya Ding - PhD Candidate, Advisors: Edward Freedman, PhD, John Foxe, PhD, and Kuan Hong Wang, PhD

CLN3 disease is one of the most prevalent forms of Neuronal Ceroid Lipofuscinosis (NCL), resulting from homozygous mutations in the human CLN3 gene. It is characterized by abnormal accumulation of ceroid lipofuscin in the lysosomes of multiple cell types, with neurons most severely affected. Individuals with CLN3 disease have early onset of blindness, motor deficits, and progressive declines in cognitive functioning and verbal intellectual abilities starting from juvenile age. However, effects of CLN3 mutations on auditory processing remain unknown. Although concurrent CLN3 mouse models successfully recapitulate ceroid lipofuscin accumulation and visual and motor deficits in humans, less is known about the link between cellular pathology and progression of disease phenotypes. Objective cross-species neurologic markers are also not well established. To fill these knowledge gaps, we will examine the effects of CLN3 mutations on auditory processing in terms of both ceroid lipofuscin accumulation and neurophysiological changes.

We hypothesize that CLN3 mutations induce cell type specific accumulation of ceroid lipofuscin and predispose neurons for neurophysiological changes and auditory processing deficits. In aim 1, we will determine whether ceroid lipofuscin preferentially accumulates in specific cell types in the CLN3 -/- mouse model. We will utilize confocal microscopy imaging of Subunit C of mitochondrial ATP Synthase (SCMAS), a common immunofluorescence marker for ceroid lipofuscin, to access accumulation of ceroid lipofuscin in different auditory processing brain regions across multiple time points. We will also test co-localization of SCMAS with parvalbumin (PV) to determine whether ceroid lipofuscin preferentially accumulates in PV + interneurons. Aim 2 will examine whether ceroid lipofuscin accumulation is associated with auditory electroencephalography (EEG) changes in the CLN3 -/- mouse model. We will conduct auditory duration mismatch negativity (MMN) to measure age-related neurophysiological changes. In aim 3, we will test whether CLN3 mutations disrupt the balance of excitatory and inhibitory neural activities in auditory processing. Fiber photometry Ca2+ imaging will be used to measure neural activity changes in PV-Cre CLN3 animals after viral injection of GCaMP6 reporter.

Overall, this project will provide a foundation to uncover longitudinal neuropathology of auditory processing in CLN3 disease and bridge the gap between current pathological and neurophysiological measurements. It will also help to develop auditory MMN as a clinically relevant biomarker and establish cross-species endophenotype.

 Nov 30, 2022 @ 1:00 p.m.
 Medical Center | Ryan Case Method Room (1-9576)

NSC 503 Seminars

Erin Murray; Michael Lim; Tanique McDonald - PhD Candidate , Neuroscience Graduate Program; PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Rick Libby & Laurel Carney

 Dec 05, 2022 @ 4:00 p.m.

NSC 503 Seminars

Daulton Myers; Mariah Marrero - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators:  Harris Gelbard & Krystel Huxlin

 Dec 12, 2022 @ 4:00 p.m.

The Elizabeth Doty Lecture: Gamma Oscillations, Long-range Inhibition And Prefrontal-Dependent Cognition

Vikaas Sohal, MD, PhD - Associate Professor, Psychiatry, Weill Institute for Neurosciences
University of California at San Francisco

 Jan 12, 2023 @ 4:00 p.m.
 Medical Center | Ryan Case Method Room (1-9576)

Host: The University of Rochester School of Medicine and Dentistry Department of Neuroscience and the Del Monte Institute for Neuroscience

NSC 503 Seminars

Caitlin Sharp; Sarah Yablonski - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Ken Henry & Manuel Gomez-Ramirez

 Jan 23, 2023 @ 4:00 p.m.

NSC 503 Seminars

Catalina Guzman; Sean Lydon - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Paul Kammermeir & Amy Kiernan

 Jan 30, 2023 @ 4:00 p.m.

NSC 503 Seminars

Leslie Gonzales; Mark Osabutey - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Anne Luebke & Chris Proschel

 Feb 06, 2023 @ 4:00 p.m.

NSC 503 Seminars

Estephanie Balbuena; Julia Granato - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Ian Fiebelkorn & Greg DeAngelis

 Feb 13, 2023 @ 4:00 p.m.

NSC 503 Seminars

Lia Calcines Rodriguez; Thomas Delgado - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Krishnan Padmanabhan & Martina Poletti

 Feb 20, 2023 @ 4:00 p.m.

NSC 503 Seminars

Abigail Alpers; Jeeyun Kim - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Ed Freedman & Marc Schieber

 Feb 27, 2023 @ 4:00 p.m.

NSC 503 Seminars

Andrea Campbell; Dominic Bunn - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Jude Mitchell & Gail Johnson

 Mar 13, 2023 @ 4:00 p.m.

NSC 503 Seminars

Michael Lim; Tanique McDonald - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Margot Mayer-Proschel & Jean Bidlack

 Mar 20, 2023 @ 4:00 p.m.

NSC 503 Seminars

Erin Murray; Mariah Marrero - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Marissa Sobolewski & Suzanne Haber

 Mar 27, 2023 @ 4:00 p.m.

Mary Notter Lectureship: TBA

Katalin Gothard, MD, PhD - Professor, Departments of Physiology and Neuroscience, University of Arizona

 Mar 30, 2023 @ 4:00 p.m.

Host: Lizabeth Romanski

NSC 503 Seminars

Daulton Myers; Margaux Masten - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Wei Hsu & David Dodell-Feder

 Apr 03, 2023 @ 4:00 p.m.

NSC 503 Seminars

Alexis Feidler - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Erika ANderson & Dragony Fu

 Apr 10, 2023 @ 4:00 p.m.

NSC 503 Seminars

Linh Le; Matt Adusei - PhD Candidate, Neuroscience Graduate Program

Faculty Evaluators: Brian Keane & Juliette McGregor

 Apr 17, 2023 @ 4:00 p.m.

TBA

Dean Salisbury, PhD - Director Clinical Neurophysiology Research Lab, Professor of Psychiatry, University of Pittsburgh

 May 18, 2023 @ 4:00 p.m.

Host: John Foxe

TBA

Yoland Smith, PhD - Division Chief, Neuropharmacology and Neurology Disease, Emory National Primate Research Center

 Jun 01, 2023 @ 4:00 p.m.

Host: John Foxe