Article
Altered intersubunit communication is the molecular basis for functional defects of pathogenic p97 mutants.
The Journal of biological chemistry - 20 Dec 2013
Tang Wai Kwan, Xia Di
Abstract excerpt
The human AAA ATPase p97 is a molecular chaperone essential in cellular proteostasis. Single amino acid substitutions in p97 have been linked to a clinical multiple-disorder condition known as inclusion body myopathy associated with Paget's disease of the bone and frontotemporal dementia. How the mutations affect the molecular mechanism that governs the function of p97 remains unclear. Here, we show that within...
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