Ubr1 - ubiquitin protein ligase E3 component n-recognin 1

Enables ubiquitin-protein transferase activity. Acts upstream of or within ubiquitin-dependent protein catabolic process. Part of proteasome complex and ubiquitin ligase complex. Is expressed in several structures, including branchial arch; genitourinary system; limb bud; liver; and lung. Used to study Johanson-Blizzard syndrome. Human ortholog(s) of this gene implicated in Johanson-Blizzard syndrome. Orthologous to human UBR1 (ubiquitin protein ligase E3 component n-recognin 1). [provided by Alliance of Genome Resources, Apr 2022]