Progressive mitochondrial dysfunction in cerebellar synaptosomes of cystatin B-deficient mice

The involvement of mitochondrial dysfunction in cystatin B (CSTB) deficiency has been suggested, but its role in the onset of neurodegeneration, myoclonus, and ataxia in the CSTB-deficient mouse model (Cstb−/−) is yet unknown. CSTB is an inhibitor of lysosomal and nuclear cysteine cathepsins. In hum...

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Bibliographic Details
Main Authors: Katarin Gorski, Christopher B. Jackson, Tuula A. Nyman, Veronika Rezov, Brendan J. Battersby, Anna-Elina Lehesjoki
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-05-01
Series:Frontiers in Molecular Neuroscience
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Online Access:https://www.frontiersin.org/articles/10.3389/fnmol.2023.1175851/full