
This gene is encodes a cytoplasmic protein with three cytoskeletal-associated protein-glycine-conserved (CAP-GLY) domains that functions as a deubiquitinating enzyme. Mutations in this gene have been associated with cylindromatosis| multiple familial trichoepithelioma| and Brooke-Spiegler syndrome. Alternate transcriptional splice variants| encoding different isoforms| have been characterized.

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