Clpp - caseinolytic mitochondrial matrix peptidase proteolytic subunit

Predicted to enable ATP-dependent peptidase activity; ATPase binding activity; and serine-type endopeptidase activity. Predicted to be involved in membrane protein proteolysis and protein quality control for misfolded or incompletely synthesized proteins. Located in mitochondrion. Is expressed in several structures, including extraembryonic component; liver; lung; pancreas; and placenta. Used to study Perrault syndrome. Human ortholog(s) of this gene implicated in Perrault syndrome. Orthologous to human CLPP (caseinolytic mitochondrial matrix peptidase proteolytic subunit). [provided by Alliance of Genome Resources, Apr 2022]