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Structural basis of impaired disaggregase function in the oxidation-sensitive SKD3 mutant causing 3-methylglutaconic aciduria

Mitochondria are critical to cellular and organismal health. To prevent damage, mitochondria have evolved protein quality control machines to survey and maintain the mitochondrial proteome. SKD3, also known as CLPB, is a ring-forming, ATP-fueled protein disaggregase essential for preserving mitochon...

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Detalles Bibliográficos
Autores principales: Lee, Sukyeong, Lee, Sang Bum, Sung, Nuri, Xu, Wendy W., Chang, Changsoo, Kim, Hyun-Eui, Catic, Andre, Tsai, Francis T. F.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10090083/
https://www.ncbi.nlm.nih.gov/pubmed/37041140
http://dx.doi.org/10.1038/s41467-023-37657-9