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Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3

The AAA+ protein, Skd3 (human CLPB), solubilizes proteins in the mitochondrial intermembrane space, which is critical for human health. Skd3 variants with defective protein-disaggregase activity cause severe congenital neutropenia (SCN) and 3-methylglutaconic aciduria type 7 (MGCA7). How Skd3 disagg...

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Detalles Bibliográficos
Autores principales: Cupo, Ryan R., Rizo, Alexandrea N., Braun, Gabriel A., Tse, Eric, Chuang, Edward, Gupta, Kushol, Southworth, Daniel R., Shorter, James
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9584538/
https://www.ncbi.nlm.nih.gov/pubmed/36170828
http://dx.doi.org/10.1016/j.celrep.2022.111408