CLPP : caseinolytic mitochondrial matrix peptidase proteolytic subunit
Description
The CLPP (caseinolytic mitochondrial matrix peptidase proteolytic subunit) is a protein-coding gene located on chromosome 19.
The CLPP gene provides instructions for making the ClpP subunit protein. Multiple copies of this protein interact to form a barrel-shaped chamber, the ClpP complex. This complex is part of the ClpXP protease, which breaks down improperly folded proteins. Another part of the ClpXP protease, the ClpX complex, unfolds abnormal proteins and feeds them into the ClpP complex for degradation. The ClpP complex is found in mitochondria, the cell's energy-producing centers.
The CLPP protein is a protease component of the Clp complex that degrades proteins and peptides in an ATP-dependent manner. It requires the presence of CLPX for optimal activity. The Clp complex has a wide range of substrates including CSN1S1, CSN2, CSN3, and synthetic peptides. It may play a general housekeeping role in the cell rather than targeting specific substrates. CLPP also degrades PINK1 in the mitochondrion.
CLPP is also known as DFNB81, PRLTS3.
