lonp-1 encodes a homolog of the Lon mitochondrial protease; loss of lonp-1 activity results in impaired proteolysis of the ATFS-1 bZip transcription factor that partitions between the mitochondria and the nucleus and functions as part of the mitochondrial unfolded protein response (UPRmt).
Predicted to enable ATP-dependent peptidase activity and single-stranded DNA binding activity. Involved in protein catabolic process. Predicted to be located in mitochondrial matrix. Expressed in head and tail. Human ortholog(s) of this gene implicated in CODAS syndrome. Is an ortholog of human LONP1 (lon peptidase 1, mitochondrial).
Map position created from combination of previous interpolated map position (based on known location of sequence) and allele information. Therefore this is not a genetic map position based on recombination frequencies or genetic experiments. This was done on advice of the CGC.