Ubiquitin-specific protease 14 is an enzyme that in humans is encoded by the USP14gene.
This gene encodes a member of the ubiquitin-specific processing (UBP) family of proteases that is a deubiquitinating enzyme (DUB) with His and Cys domains. This protein is located in the cytoplasm and cleaves the ubiquitinmoiety from ubiquitin-fused precursors and ubiquitinylated proteins. Mice with a mutation that results in reduced expression of the ortholog of this protein are retarded for growth, develop severe tremors by 2 to 3 weeks of age followed by hindlimb paralysis and death by 6 to 10 weeks of age. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.
Wilson SM, Bhattacharyya B, Rachel RA, Coppola V, Tessarollo L, Householder DB, Fletcher CF, Miller RJ, Copeland NG, Jenkins NA (2002). "Synaptic defects in ataxia mice result from a mutation in Usp14, encoding a ubiquitin-specific protease.". Nature Genetics. 32 (3): 420–425. doi:10.1038/ng1006. PMID12368914.
Shinji S, Naito Z, Ishiwata S, Ishiwata T, Tanaka N, Furukawa K, Suzuki H, Seya T, Matsuda A, Katsuta M, Tajiri T (2006). "Ubiquitin-specific protease 14 expression in colorectal cancer is associated with liver and lymph node metastases.". Oncol. Rep. 15 (3): 539–43. doi:10.3892/or.15.3.539. PMID16465409.
Crimmins S, Jin Y, Wheeler C, Huffman AK, Chapman C, Dobrunz LE, Levey A, Roth KA, Wilson JA, Wilson SM (2006). "Transgenic rescue of ataxia mice with neuronal-specific expression of ubiquitin-specific protease 14.". J. Neurosci. 26 (44): 11423–31. doi:10.1523/JNEUROSCI.3600-06.2006. PMID17079671.