The N-end rule pathway is one proteolytic pathway of the
ubiquitin system. The recognition component of this pathway,
encoded by this gene, binds to a destabilizing N-terminal residue
of a substrate protein and participates in the formation of a
substrate-linked multiubiquitin chain. This leads to the eventual
degradation of the substrate protein. The protein described in this
record has a RING-type zinc finger and a UBR-type zinc finger.
Mutations in this gene have been associated with Johanson-Blizzard
syndrome.
