Protease that specifically cleaves 'Lys-63'-linked polyubiquitin chains. Has endodeubiquitinase activity. Plays an important role in the regulation of pathways leading to NF-kappa-B activation. Contributes to the regulation of cell survival, proliferation and differentiation via its effects on NF-kappa-B activation. Negative regulator of Wnt signaling. Inhibits HDAC6 and thereby promotes acetylation of alpha-tubulin and stabilization of microtubules. Plays a role in the regulation of microtubule dynamics, and thereby contributes to the regulation of cell proliferation, cell polarization, cell migration, and angiogenesis. Required for normal cell cycle progress and normal cytokinesis. Inhibits nuclear translocation of NF-kappa-B. Plays a role in the regulation of inflammation and the innate immune response, via its effects on NF-kappa-B activation. Dispensable for the maturation of intrathymic natural killer cells, but required for the continued survival of immature natural killer cells. Negatively regulates TNFRSF11A signaling and osteoclastogenesis (By similarity).

• GO:0007049 : cell cycle
• GO:0006917 : induction of apoptosis
• GO:0045087 : innate immune response
• GO:0090090 : negative regulation of canonical Wnt receptor signaling pathway
• GO:0042347 : negative regulation of NF-kappaB import into nucleus
• GO:0032088 : negative regulation of NF-kappaB transcription factor activity
• GO:0032480 : negative regulation of type I interferon production
• GO:0070423 : nucleotide-binding oligomerization domain containing signaling pathway
• GO:2001238 : positive regulation of extrinsic apoptotic signaling pathway
• GO:0070536 : protein K63-linked deubiquitination
• GO:0070507 : regulation of microtubule cytoskeleton organization
• GO:0007346 : regulation of mitotic cell cycle
• GO:0006511 : ubiquitin-dependent protein catabolic process
• GO:0016055 : Wnt receptor signaling pathway

Source: The Gene Ontology Consortium. Gene ontology: tool for the unification of biology. Nat. Genet.. May 2000;25(1):25-9.
World Wide Web URL: http://www.geneontology.org/

• OMIM:605018 : Cylindromatosis, familial (OMIM:132700 )
• OMIM:605018 : Brooke-Spiegler syndrome (OMIM:605041 )
• OMIM:605018 : Trichoepithelioma, multiple familial, 1 (OMIM:601606 )

Source: Online Mendelian Inheritance in Man, OMIM®. McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD), May, 2012.
World Wide Web URL: http://omim.org/