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...

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Bibliographic Details
Published in:Sci Rep
Main Authors: Wang, Jinliang, Enriquez, Adrian S., Li, Jihui, Rodriguez, Alejandro, Holguin, Bianka, Von Salzen, Daniel, Bhatt, Jay M., Bernal, Ricardo A.
Format: Artigo
Language:Inglês
Published: Nature Publishing Group UK 2019
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Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC6707239/
https://ncbi.nlm.nih.gov/pubmed/31444388
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-019-48762-5
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