AUTHOR=Huang Liqin , Zhang Zhaohui
TITLE=CSPα in neurodegenerative diseases
JOURNAL=Frontiers in Aging Neuroscience
VOLUME=14
YEAR=2022
URL=https://www.frontiersin.org/journals/aging-neuroscience/articles/10.3389/fnagi.2022.1043384
DOI=10.3389/fnagi.2022.1043384
ISSN=1663-4365
ABSTRACT=
Adult-onset neuronal ceroid lipofuscinosis (ANCL) is a rare neurodegenerative disease characterized by epilepsy, cognitive degeneration, and motor disorders caused by mutations in the DNAJC5 gene. In addition to being associated with ANCL disease, the cysteine string proteins α (CSPα) encoded by the DNAJC5 gene have been implicated in several neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD), and Huntington's disease. However, the pathogenic mechanism responsible for these neurodegenerative diseases has not yet been elucidated. Therefore, this study examines the functional properties of the CSPα protein and the related mechanisms of neurodegenerative diseases.