Paul J Mathews

Photo of Paul J Mathews
Title(s)Associate Professor-in-Residence, Neurology
SchoolMedicine
ORCID ORCID Icon0000-0002-1991-0798 Additional info
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    Paul J. Mathews, PhD, is an Associate Professor-in-Residence in the UCLA Department of Neurology and an Investigator and member of the Board of Directors at The Lundquist Institute. His research integrates systems neuroscience and therapeutic development to understand cerebellar function in health and disease.

    Dr. Mathews studies how communication between the cerebellum and forebrain contributes to behavioral flexibility, learning, and social behavior. His research uses electrophysiology, neuroanatomy, quantitative behavioral testing, and targeted manipulation of specific cells and circuits. In collaboration with Neil Harris, PhD, he also uses functional ultrasound imaging to investigate how traumatic brain injury alters brain-wide functional connectivity and how these network-level changes evolve with injury and recovery.

    A major focus of Dr. Mathews’ research is Ataxia-Telangiectasia (A-T), a rare genetic disorder that causes progressive cerebellar degeneration. His group developed a clinically relevant ataxic mouse model of A-T and uses it to investigate Purkinje neuron vulnerability, altered neuronal function, and disease progression. His translational research seeks to restore ATM production and function through approaches that include small-molecule readthrough, base editing, and prime editing. These strategies are evaluated in patient-derived cells and animal models using molecular, physiological, histological, and behavioral outcomes. His goal is to connect fundamental neuroscience with therapeutic development to advance treatments for A-T and other rare neurogenetic diseases.

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    University of Texas, Austin, TXPhD12/2008Neuroscience
    University of Oregon, Eugene, ORBS07/2001Biology

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    Early In Vivo Testing of Precision Gene Editing Therapies for Ataxia-Telangiectasia
    Action for A-T/ATCP Oct 1, 2026 - Sep 30, 2029
    Description: The overall goal of this project is to conduct early in vivo testing of precision gene editing as a potential therapeutic strategy for ataxia-telangiectasia (A-T). The funded proposal was designed to evaluate whether gene editing approaches could correct a disease-causing Atm mutation in a relevant A-T mouse model and to determine the most promising delivery and analysis strategy for subsequent efficacy studies.
    Gene Editing for A-T
    A-T Children's Project Oct 1, 2025 - Dec 1, 2026
    Role: PI
    Cerebellar Neuromodulation to Reduce Cognitive Dysfunction After Multiple Subconcussive Impacts
    DOD GRANT14267287Dec 10, 2024 - Dec 11, 2027
    Role: Co-Investigator
    Description: The Principal Investigator (PI) of this application proposes to obtain a mechanistic understanding of the effect of cognitive task burden on brain functional connectivity after traumatic brain injury (TBI). The specific aims are to (1) determine the effect of successive impacts on neural circuits related to cognition; (2) determine the effect of cerebellar neuromodulation on cognition; and (3) investigate clinical translatability.
    Defining cerebellar pathophysiology in Ataxia Telangiectasia
    NIH R01NS131646Jan 15, 2024 - Dec 31, 2028
    Role: Principal Investigator
    Behavioral and brain network effects of dysfunction in the cognitive cerebellum
    NIH R21MH126359Jul 1, 2022 - Jun 30, 2025
    Role: Principal Investigator
    Behavioral and brain network effects of dysfunction in the cognitive cerebellum
    NIMH R21MH126359Jul 1, 2022 - Jun 30, 2023
    Role: PI
    Optimizing Small Molecule Read-Through Compounds for Treating AtaxiaTelangiectasia
    NINDS R61NS119708Apr 19, 2022 - Mar 1, 2024
    Role: PI
    Ataxia Telangiectasia: Elucidating Disease Pathogenesis and Testing New Treatments
    NIH R21NS108117Jun 15, 2018 - May 31, 2021
    Role: Principal Investigator
    A New Mouse Model of Ataxia-Telangiectasia for Testing Therapeutic Read-Through Compounds
    NIH R03NS103066Jan 15, 2018 - Dec 31, 2019
    Role: Principal Investigator
    An optogenetic approach to exploring climbing fiber connections in the cerebellum
    NIH F32NS074719Aug 28, 2011 - Aug 27, 2014
    Role: Principal Investigator
    Synaptic Integration in the Medial Superior Olive
    NIH F31DC008030Apr 28, 2006 - Apr 27, 2009
    Role: Principal Investigator

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    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help. to make corrections and additions.
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    Altmetrics Details PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Convergent and Divergent Cerebellar Alterations in 22q11.2 Copy Number Variants. Biol Psychiatry Glob Open Sci. 2026 Jul; 6(4):100718. Fung H, O'Hora KP, Boen R, Wang HR, Amir CM, Schleifer CH, Kushan-Wells L, Miranda BA, Bondy EK, Mathews PJ, Bearden CE. PMID: 42058778; PMCID: PMC13122804.
      View in: PubMed   Mentions:
    2. Correlated signatures of social behavior in cerebellum and anterior cingulate cortex. Elife. 2024 02 12; 12. Hur SW, Safaryan K, Yang L, Blair HT, Masmanidis SC, Mathews PJ, Aharoni D, Golshani P. PMID: 38345922; PMCID: PMC10942583.
      View in: PubMed   Mentions: 9     Fields:    Translation:Animals
    3. Correlated signatures of social behavior in cerebellum and anterior cingulate cortex. bioRxiv. 2023 Oct 14. Hur SW, Safaryan K, Yang L, Blair HT, Masmanidis SC, Mathews PJ, Aharoni D, Golshani P. PMID: 37066345; PMCID: PMC10104017.
      View in: PubMed   Mentions:
    4. Editorial: Unravelling the complex and multifaceted role of the cerebellum in health and disease. Front Syst Neurosci. 2023; 17:1155939. Mathews PJ, Paradis AL, Cvetanovic M, Carlson ES, Parker KL. PMID: 37034016; PMCID: PMC10076819.
      View in: PubMed   Mentions: 1  
    5. A novel, ataxic mouse model of ataxia telangiectasia caused by a clinically relevant nonsense mutation. Elife. 2021 11 01; 10. Perez H, Abdallah MF, Chavira JI, Norris AS, Egeland MT, Vo KL, Buechsenschuetz CL, Sanghez V, Kim JL, Pind M, Nakamura K, Hicks GG, Gatti RA, Madrenas J, Iacovino M, McKinnon PJ, Mathews PJ. PMID: 34723800; PMCID: PMC8601662.
      View in: PubMed   Mentions: 12     Fields:    Translation:AnimalsCells
    6. Optogenetic fMRI and electrophysiological identification of region-specific connectivity between the cerebellar cortex and forebrain. Neuroimage. 2018 06; 173:370-383. Choe KY, Sanchez CF, Harris NG, Otis TS, Mathews PJ. PMID: 29496611; PMCID: PMC5911204.
      View in: PubMed   Mentions: 26     Fields:    Translation:Animals
    7. α7-Nicotinic acetylcholine receptor inhibition by indinavir: implications for cognitive dysfunction in treated HIV disease. AIDS. 2017 05 15; 31(8):1083-1089. Ekins S, Mathews P, Saito EK, Diaz N, Naylor D, Chung J, McMurtray AM. PMID: 28358738.
      View in: PubMed   Mentions: 10     Fields:    Translation:AnimalsCells
    8. Enabling Anyone to Translate Clinically Relevant Ideas to Therapies. Pharm Res. 2017 01; 34(1):1-6. Ekins S, Diaz N, Chung J, Mathews P, McMurtray A. PMID: 27620174.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansAnimals
    9. Circuit mechanisms underlying motor memory formation in the cerebellum. Neuron. 2015 Apr 22; 86(2):529-40. Lee KH, Mathews PJ, Reeves AM, Choe KY, Jami SA, Serrano RE, Otis TS. PMID: 25843404; PMCID: PMC4417109.
      View in: PubMed   Mentions: 87     Fields:    Translation:AnimalsCells
    10. Effects of climbing fiber driven inhibition on Purkinje neuron spiking. J Neurosci. 2012 Dec 12; 32(50):17988-97. Mathews PJ, Lee KH, Peng Z, Houser CR, Otis TS. PMID: 23238715; PMCID: PMC3532857.
      View in: PubMed   Mentions: 60     Fields:    Translation:AnimalsCells
    11. How do climbing fibers teach? Front Neural Circuits. 2012; 6:95. Otis TS, Mathews PJ, Lee KH, Maiz J. PMID: 23226116; PMCID: PMC3510640.
      View in: PubMed   Mentions: 8     Fields:    
    12. Control of submillisecond synaptic timing in binaural coincidence detectors by K(v)1 channels. Nat Neurosci. 2010 May; 13(5):601-9. Mathews PJ, Jercog PE, Rinzel J, Scott LL, Golding NL. PMID: 20364143; PMCID: PMC3375691.
      View in: PubMed   Mentions: 111     Fields:    Translation:AnimalsCells
    13. Perisomatic voltage-gated sodium channels actively maintain linear synaptic integration in principal neurons of the medial superior olive. J Neurosci. 2010 Feb 10; 30(6):2039-50. Scott LL, Mathews PJ, Golding NL. PMID: 20147532; PMCID: PMC2827923.
      View in: PubMed   Mentions: 45     Fields:    Translation:AnimalsCells
    14. Posthearing developmental refinement of temporal processing in principal neurons of the medial superior olive. J Neurosci. 2005 Aug 31; 25(35):7887-95. Scott LL, Mathews PJ, Golding NL. PMID: 16135745; PMCID: PMC6725447.
      View in: PubMed   Mentions: 102     Fields:    Translation:AnimalsCells
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