Role of UCHL1 in the pathogenesis of neurodegenerative diseases and brain injury.

Ageing Res Rev

Departments of Neurology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15213, United States; Geriatric Research Education and Clinical Center, VA Pittsburgh Healthcare System, Pittsburgh, PA 15213, United States. Electronic address:

Published: April 2023

UCHL1 is a multifunctional protein expressed at high concentrations in neurons in the brain and spinal cord. UCHL1 plays important roles in regulating the level of cellular free ubiquitin and redox state as well as the degradation of select proteins. This review focuses on the potential role of UCHL1 in the pathogenesis of neurodegenerative diseases and brain injury and recovery. Subjects addressed in the review include 1) Normal physiological functions of UCHL1. 2) Posttranslational modification sites and splice variants that alter the function of UCHL1 and mouse models with mutations and deletions of UCHL1. 3) The hypothesized role and pathogenic mechanisms of UCHL1 in neurodegenerative diseases and brain injury. 4) Potential therapeutic strategies targeting UCHL1 in these disorders.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9992267PMC
http://dx.doi.org/10.1016/j.arr.2023.101856DOI Listing

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