Article
Autocatalytic processing of m-AAA protease subunits in mitochondria.
Molecular biology of the cell - 1 Oct 2009
Koppen Mirko, Bonn Florian, Ehses Sarah, Langer Thomas
Abstract excerpt
m-AAA proteases are ATP-dependent proteolytic machines in the inner membrane of mitochondria which are crucial for the maintenance of mitochondrial activities. Conserved nuclear-encoded subunits, termed paraplegin, Afg3l1, and Afg3l2, form various isoenzymes differing in their subunit composition in mammalian mitochondria. Mutations in different m-AAA protease subunits are associated with distinct neuronal...
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