Automated description from the Alliance of Genome Resources (Release 7.0.0)
Enables several functions, including protein homodimerization activity; ubiquitin protein ligase activity; and zinc ion binding activity. Involved in several processes, including V(D)J recombination; lymphocyte differentiation; and protein autoubiquitination. Acts upstream of or within several processes, including T cell homeostasis; negative regulation of thymocyte apoptotic process; and thymus development. Located in nucleus. Is expressed in several structures, including early conceptus; gut; hemolymphoid system; mesenchyme derived from splanchnopleure; and metanephros. Human ortholog(s) of this gene implicated in Omenn syndrome; combined cellular and humoral immune defects with granulomas; severe combined immunodeficiency; and severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, Nk cell-positive. Orthologous to human RAG1 (recombination activating 1).