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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 h...

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Detalles Bibliográficos
Autores principales: Gorski, Katarin, Jackson, Christopher B., Nyman, Tuula A., Rezov, Veronika, Battersby, Brendan J., Lehesjoki, Anna-Elina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10213208/
https://www.ncbi.nlm.nih.gov/pubmed/37251643
http://dx.doi.org/10.3389/fnmol.2023.1175851