[HTML][HTML] The MitCHAP-60 disease is due to entropic destabilization of the human mitochondrial Hsp60 oligomer
A Parnas, M Nadler, S Nisemblat, A Horovitz… - Journal of biological …, 2009 - ASBMB
The 60-kDa heat shock protein (mHsp60) is a vital cellular complex that mediates the folding
of many of the mitochondrial proteins. Its function is executed in cooperation with the co-
chaperonin, mHsp10, and requires ATP. Recently, the discovery of a new mHsp60-
associated neurodegenerative disorder, MitCHAP-60 disease, has been reported. The
disease is caused by a point mutation at position 3 (D3G) of the mature mitochondrial Hsp60
protein, which renders it unable to complement the deletion of the homologous bacterial …
of many of the mitochondrial proteins. Its function is executed in cooperation with the co-
chaperonin, mHsp10, and requires ATP. Recently, the discovery of a new mHsp60-
associated neurodegenerative disorder, MitCHAP-60 disease, has been reported. The
disease is caused by a point mutation at position 3 (D3G) of the mature mitochondrial Hsp60
protein, which renders it unable to complement the deletion of the homologous bacterial …