Presenilins-associated rhomboid-like protein, mitochondrial

Function

• control of apoptosis during postnatal growth
• proteolytic processing of an antiapoptotic form of OPA1 which prevents the release of mitochondrial cytochrome C in response to intrinsic apoptoptic signals
• cleaves type-1 transmembrane domains using a catalytic triad composed of Ser, His & Asp (charge-relay system) contributed by different transmembrane domains
• interacts with PSEN1 & PSEN2
• binds OPA1
• contains Pbeta
  • formed via PARL-dependent proteolytic processing (beta-cleavage) & translocated into the nucleus by an unknown mechanism

Compartment

• mitochondrial inner membrane
• Pbeta is translocated into the nucleus

More general terms

• serine protease
• mitochondrial membrane protein
• transmembrane 7c protein

Database

• OMIM: https://mirror.omim.org/entry/607858
• UniProt: http://www.uniprot.org/uniprot/Q9H300.html