lon protease homolog, mitochondrial; lon protease-like protein; LONP; LONHs; mitochondrial ATP-dependent protease lon; serine protease 15 (LONP1, PRSS15)

Function

• required for intramitochondrial proteolysis
• catalyzes initial steps of protein degradation
• hydrolyzes ATP & degrades protein & peptide substrates (ATP-dependent)
• binds to single-stranded but not to double-stranded DNA

Structure

• belongs to the peptidase S16 family
• contains 1 lon domain

Compartment

mitochondrial matrix

Expression

• expressed in duodenum, heart, lung & liver
• not expressed in thymus

More general terms

• serine protease
• mitochondrial protein

Database

• UniProt: http://www.uniprot.org/uniprot/P36776.html
• OMIM: https://mirror.omim.org/entry/605490