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MitCHAP-60 and Hereditary Spastic Paraplegia SPG-13 Arise from an Inactive hsp60 Chaperonin that Fails to Fold the ATP Synthase β-Subunit
The human mitochondrial heat shock protein 60 (hsp60) is a tetradecameric chaperonin that folds proteins in the mitochondrial matrix. An hsp60 D3G mutation leads to MitCHAP-60, an early onset neurodegenerative disease while hsp60 V72I has been linked to SPG13, a form of hereditary spastic paraplegia...
Autores principales: | Wang, Jinliang, Enriquez, Adrian S., Li, Jihui, Rodriguez, Alejandro, Holguin, Bianka, Von Salzen, Daniel, Bhatt, Jay M., Bernal, Ricardo A. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6707239/ https://www.ncbi.nlm.nih.gov/pubmed/31444388 http://dx.doi.org/10.1038/s41598-019-48762-5 |
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