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ubiquitin C-terminal hydrolase; UCH; ubiquitin thiolesterase; ubiquitin-specific processing protease; deubiquitinating enzyme

Function: - protease specific for ubiquitinated proteins - at least 4 ubiquitins attached to a single protein site is needed for ubiquitin mediated proteolysis - regulatory role at the level of protein turnover by preventing degradation Pathology: - defect or alteration in the expression of enzymes catalyzing the removal of ubiquitin from ubiquitin-proteins conjugates may be a characteristic of neurodegenerative diseases such as Alzheimer disease, Parkinson disease, progressive supranuclear palsy, Pick disease & Kuf disease [3] Laboratory: - ubiquitin C-terminal hydrolase in blood FDA-approved to evaluate mild traumatic brain injury (concussion) in adults - assessment of need for CT of brain [5]

Related

ubiquitin (UBCEP2, UBB, UBC)

Specific

ataxin-3; Machado-Joseph disease protein 1; spinocerebellar ataxia type 3 protein (ATXN3, ATX3, MJD, MJD1, SCA3) inactive ubiquitin carboxyl-terminal hydrolase 17-like protein 4 (USP17L4) inactive ubiquitin carboxyl-terminal hydrolase 17-like protein 7 (USP17L7) inactive ubiquitin carboxyl-terminal hydrolase 17-like protein 8 (USP17L8) inactive ubiquitin carboxyl-terminal hydrolase 50; inactive ubiquitin-specific peptidase 50 (USP50) inactive ubiquitin carboxyl-terminal hydrolase 54 (inactive ubiquitin-specific peptidase 54, USP54, C10orf29) ubiquitin carboxyl-terminal hydrolase 1; deubiquitinating enzyme 1; hUBP; ubiquitin thiolesterase 1; ubiquitin-specific-processing protease 1 (USP1) ubiquitin carboxyl-terminal hydrolase 2; 41 kD ubiquitin-specific protease; deubiquitinating enzyme 2; ubiquitin thiolesterase 2; ubiquitin-specific-processing protease 2 (USP2, UBP41) ubiquitin carboxyl-terminal hydrolase 3; deubiquitinating enzyme 3; ubiquitin thiolesterase 3; ubiquitin-specific-processing protease 3 (USP3) ubiquitin carboxyl-terminal hydrolase 4; deubiquitinating enzyme 4; ubiquitin thiolesterase 4; ubiquitin-specific-processing protease 4; ubiquitous nuclear protein homolog (USP4, UNP, UNPH) ubiquitin carboxyl-terminal hydrolase 5; deubiquitinating enzyme 5; isopeptidase T; ubiquitin thiolesterase 5; ubiquitin-specific-processing protease 5 (USP5, ISOT) ubiquitin carboxyl-terminal hydrolase 6 (ubiquitin C terminal hydrolase TRE2, ubiquitin thioesterase 6, ubiquitin-specific-processing protease 6, deubiquitinating enzyme 6, proto-oncogene TRE-2, USP6, HRP1, TRE2) ubiquitin carboxyl-terminal hydrolase 7; deubiquitinating enzyme 7; Herpesvirus-associated ubiquitin-specific protease; ubiquitin thioesterase 7; ubiquitin-specific-processing protease 7 (USP7, HAUSP) ubiquitin carboxyl-terminal hydrolase 8 (ubiquitin thioesterase 8, ubiquitin-specific-processing protease 8, deubiquitinating enzyme 8, hUBPy, USP8, KIAA0055, UBPY) ubiquitin carboxyl-terminal hydrolase 10; deubiquitinating enzyme 10; ubiquitin thiolesterase 10; ubiquitin-specific-processing protease 10 (USP10, KIAA0190) ubiquitin carboxyl-terminal hydrolase 11; deubiquitinating enzyme 11; ubiquitin thiolesterase 11; ubiquitin-specific-processing protease 11 (USP11, UHX1) ubiquitin carboxyl-terminal hydrolase 12; deubiquitinating enzyme 12; ubiquitin thiolesterase 12; ubiquitin-hydrolyzing enzyme 1; ubiquitin-specific-processing protease 12 (USP12, UBH1, USP12L1) ubiquitin carboxyl-terminal hydrolase 13; deubiquitinating enzyme 13; isopeptidase T-3; ISOT-3; ubiquitin thiolesterase 13; ubiquitin-specific-processing protease 13 (USP13, ISOT3) ubiquitin carboxyl-terminal hydrolase 14; deubiquitinating enzyme 14; ubiquitin thiolesterase 14; ubiquitin-specific-processing protease 14 (USP14, TGT) ubiquitin carboxyl-terminal hydrolase 15; deubiquitinating enzyme 15; ubiquitin thiolesterase 15; ubiquitin-specific-processing protease 15; Unph-2; Unph4 (USP15, KIAA0529) ubiquitin carboxyl-terminal hydrolase 16; deubiquitinating enzyme 16; ubiquitin thiolesterase 16; ubiquitin-processing protease UBP-M; ubiquitin-specific-processing protease 16 (USP16) ubiquitin carboxyl-terminal hydrolase 17-like protein 10 (USP17L10) ubiquitin carboxyl-terminal hydrolase 17-like protein 11 (USP17L11) ubiquitin carboxyl-terminal hydrolase 17-like protein 12 (USP17L12) ubiquitin carboxyl-terminal hydrolase 17-like protein 13 (USP17L13) ubiquitin carboxyl-terminal hydrolase 17-like protein 15 (USP17L15) ubiquitin carboxyl-terminal hydrolase 17-like protein 17 (USP17L17) ubiquitin carboxyl-terminal hydrolase 17-like protein 18 (USP17L18) ubiquitin carboxyl-terminal hydrolase 17-like protein 19 (USP17L19) ubiquitin carboxyl-terminal hydrolase 17-like protein 2 (ubiquitin thioesterase 17-like protein 2, ubiquitin-specific-processing protease 17-like protein 2, deubiquitinating enzyme 17-like protein 2, deubiquitinating protein 3, DUB-3, USP17L2, DUB3) ubiquitin carboxyl-terminal hydrolase 17-like protein 20 (USP17L20) ubiquitin carboxyl-terminal hydrolase 17-like protein 21 (USP17L21) ubiquitin carboxyl-terminal hydrolase 17-like protein 22 (USP17L22) ubiquitin carboxyl-terminal hydrolase 17-like protein 23 (USP17L23) ubiquitin carboxyl-terminal hydrolase 17-like protein 3 (ubiquitin thioesterase 17-like protein 3, ubiquitin-specific-processing protease 17-like protein 3, deubiquitinating enzyme 17-like protein 3, USP17L3, USP17B, USP17F) ubiquitin carboxyl-terminal hydrolase 17-like protein 5 (ubiquitin thioesterase 17-like protein 5, ubiquitin-specific-processing protease 17-like protein 5, deubiquitinating enzyme 17-like protein 5, USP17L5) ubiquitin carboxyl-terminal hydrolase 17-like protein 6 (ubiquitin thioesterase 17-like protein 6, ubiquitin-specific-processing protease 17-like protein 6, deubiquitinating enzyme 17-like protein 6, USP17L6, USP17C, USP17D, USP17N) ubiquitin carboxyl-terminal hydrolase 17-like protein; ubiquitin thioesterase 17-like; ubiquitin-specific-processing protease 17-like; deubiquitinating enzyme 17-like (USP17L, USP17L1P) ubiquitin carboxyl-terminal hydrolase 17; ubiquitin thioesterase 17; ubiquitin-specific-processing protease 17; deubiquitinating enzyme 17; ubiquitin carboxyl-terminal hydrolase 17-like protein 24 (USP17, USP17L24, USP17H, USP17I, USP17J, USP17K, USP17M, USP17L25, USP17L26, USP17L27, USP17L28, USP17L29, USP17L30) ubiquitin carboxyl-terminal hydrolase 19; deubiquitinating enzyme 19; ubiquitin thiolesterase 19; ubiquitin-specific-processing protease 19; zinc finger MYND domain-containing protein 9 (USP19, KIAA0891, ZMYND9) ubiquitin carboxyl-terminal hydrolase 20; deubiquitinating enzyme 20; ubiquitin thiolesterase 20; ubiquitin-specific-processing protease 20; VHL-interacting deubiquitinating enzyme 2; hVDU2 (USP20, KIAA1003, LSFR3A, VDU2) ubiquitin carboxyl-terminal hydrolase 21; deubiquitinating enzyme 21; ubiquitin thiolesterase 21; ubiquitin-specific-processing protease 21 (USP21, PP1490) ubiquitin carboxyl-terminal hydrolase 22; deubiquitinating enzyme 22; ubiquitin thiolesterase 22; ubiquitin-specific-processing protease 22 (USP22, KIAA1063) ubiquitin carboxyl-terminal hydrolase 23 (ubiquitin-specific protease-23, ubiquitin thiolesterase 23, deubiquitinating enzyme 23, USP23) ubiquitin carboxyl-terminal hydrolase 24; deubiquitinating enzyme 24; ubiquitin thiolesterase 24; ubiquitin-specific-processing protease 24 (USP24, KIAA1057) ubiquitin carboxyl-terminal hydrolase 25; deubiquitinating enzyme 25; USP on chromosome 21; ubiquitin thiolesterase 25; ubiquitin-specific-processing protease 25 (USP25) ubiquitin carboxyl-terminal hydrolase 26; deubiquitinating enzyme 26; ubiquitin thiolesterase 26; ubiquitin-specific-processing protease 26 (USP26) ubiquitin carboxyl-terminal hydrolase 27; deubiquitinating enzyme 27; ubiquitin carboxyl-terminal hydrolase 22-like; ubiquitin thiolesterase 27; ubiquitin-specific-processing protease 27; X-linked ubiquitin carboxyl-terminal hydrolase 27 (USP27X, USP22L, USP27) ubiquitin carboxyl-terminal hydrolase 28; deubiquitinating enzyme 28; ubiquitin thiolesterase 28; ubiquitin-specific-processing protease 28 (USP28, KIAA1515) ubiquitin carboxyl-terminal hydrolase 29; deubiquitinating enzyme 29; ubiquitin thiolesterase 29; ubiquitin-specific-processing protease 29 (USP29) ubiquitin carboxyl-terminal hydrolase 30; deubiquitinating enzyme 30; ubiquitin thiolesterase 30; ubiquitin-specific-processing protease 30; Ub-specific protease 30 (USP30) ubiquitin carboxyl-terminal hydrolase 31; deubiquitinating enzyme 31; ubiquitin thiolesterase 31; ubiquitin-specific-processing protease 31 (USP31, KIAA1203) ubiquitin carboxyl-terminal hydrolase 32; deubiquitinating enzyme 32; renal carcinoma antigen NY-REN-60; ubiquitin thiolesterase 32; ubiquitin-specific-processing protease 32 (USP32, USP10) ubiquitin carboxyl-terminal hydrolase 33; deubiquitinating enzyme 33; ubiquitin thiolesterase 33; ubiquitin-specific-processing protease 33; VHL-interacting deubiquitinating enzyme 1; hVDU1 (USP33, KIAA1097, VDU1) ubiquitin carboxyl-terminal hydrolase 34 (ubiquitin thioesterase 34, ubiquitin-specific-processing protease 34, deubiquitinating enzyme 34, USP34, KIAA0570, KIAA0729) ubiquitin carboxyl-terminal hydrolase 35; deubiquitinating enzyme 35; ubiquitin thiolesterase 35; ubiquitin-specific-processing protease 35 (USP35, KIAA1372) ubiquitin carboxyl-terminal hydrolase 36; deubiquitinating enzyme 36; ubiquitin thiolesterase 36; ubiquitin-specific-processing protease 36 (USP36, KIAA1453) ubiquitin carboxyl-terminal hydrolase 37; deubiquitinating enzyme 37; ubiquitin thiolesterase 37; ubiquitin-specific-processing protease 37 (USP37, KIAA1594) ubiquitin carboxyl-terminal hydrolase 38; deubiquitinating enzyme 38; HP43.8KD; ubiquitin thiolesterase 38; ubiquitin-specific-processing protease 38 (USP38, KIAA1891) ubiquitin carboxyl-terminal hydrolase 39 (ubiquitin-specific protease-39, USP39) ubiquitin carboxyl-terminal hydrolase 40; deubiquitinating enzyme 40; ubiquitin thiolesterase 40; ubiquitin-specific-processing protease 40 (USP40) ubiquitin carboxyl-terminal hydrolase 41; deubiquitinating enzyme 41; ubiquitin thiolesterase 41; ubiquitin-specific-processing protease 41 (USP41) ubiquitin carboxyl-terminal hydrolase 42; deubiquitinating enzyme 42; ubiquitin thiolesterase 42; ubiquitin-specific-processing protease 42 (USP42) ubiquitin carboxyl-terminal hydrolase 43; deubiquitinating enzyme 43; ubiquitin thiolesterase 43; ubiquitin-specific-processing protease 43 (USP43) ubiquitin carboxyl-terminal hydrolase 44; deubiquitinating enzyme 44; ubiquitin thiolesterase 44; ubiquitin-specific-processing protease 44 (USP44) ubiquitin carboxyl-terminal hydrolase 45; deubiquitinating enzyme 45; ubiquitin thiolesterase 45; ubiquitin-specific-processing protease 45 (USP45) ubiquitin carboxyl-terminal hydrolase 46; deubiquitinating enzyme 46; ubiquitin thiolesterase 46; ubiquitin-specific-processing protease 46 (USP46) ubiquitin carboxyl-terminal hydrolase 47; deubiquitinating enzyme 47; ubiquitin thiolesterase 47; ubiquitin-specific-processing protease 47 (USP47) ubiquitin carboxyl-terminal hydrolase 48 (ubiquitin thioesterase 48, ubiquitin-specific-processing protease 48, deubiquitinating enzyme 48, USP48, USP31) ubiquitin carboxyl-terminal hydrolase 49; deubiquitinating enzyme 49; ubiquitin thiolesterase 49; ubiquitin-specific-processing protease 49 (USP49) ubiquitin carboxyl-terminal hydrolase 51; deubiquitinating enzyme 51; ubiquitin thiolesterase 51; ubiquitin-specific-processing protease 51 (USP51) ubiquitin carboxyl-terminal hydrolase BAP1; BRCA1-associated protein 1; cerebral protein 6 (BAP1, KIAA0272, hucep-6) ubiquitin carboxyl-terminal hydrolase CYLD; deubiquitinating enzyme CYLD; ubiquitin thiolesterase CYLD; ubiquitin-specific-processing protease CYLD (CYLD, CYLD1, KIAA0849, HSPC057) ubiquitin carboxyl-terminal hydrolase FAF-X; deubiquitinating enzyme FAF-X; fat facets in mammals; hFAM; fat facets protein-related, X-linked; ubiquitin thiolesterase FAF-X; ubiquitin-specific protease 9, X chromosome; ubiquitin-specific-processing protease FAF-X (USP9X, DFFRX, FAM, USP9) ubiquitin carboxyl-terminal hydrolase FAF-Y; deubiquitinating enzyme FAF-Y; fat facets protein-related, Y-linked; ubiquitin thiolesterase FAF-Y; ubiquitin-specific protease 9, Y chromosome; ubiquitin-specific-processing protease FAF-Y (USP9Y, DFFRY) ubiquitin thioesterase OTUB1; deubiquitinating enzyme OTUB1; OTU domain-containing ubiquitin aldehyde-binding protein 1; otubain-1; hOTU1; ubiquitin-specific-processing protease OTUB1 (OTUB1, OTB1, OTU1, HSPC263) ubiquitin thioesterase OTUB2; deubiquitinating enzyme OTUB2; OTU domain-containing ubiquitin aldehyde-binding protein 2; otubain-2; ubiquitin-specific-processing protease OTUB2 (OTUB2, C14orf137, OTB2, OTU2) ubiquitin thiolesterase L1; ubiquitin C-terminal hydrolase L1; UCH-L1; neuron cytoplasmic protein 9.5 (UCHL1, PARK5) ubiquitin thiolesterase L3; ubiquitin C-terminal hydrolase L3; UCH-L3 (UCHL3) ubiquitin thiolesterase L5; ubiquitin C-terminal hydrolase L5; UCH-L5 (UCHL5, UCH37, CGI-70, AD-019) ubiquitin-specific protease-3 like (USP3L) Ubl carboxyl-terminal hydrolase 18; 43 kD ISG15-specific protease; hUBP43; ISG15-specific-processing protease; Ubl thiolesterase 18 (USP18 ISG43)

General

cysteine proteinase; thiol proteinase

Properties

MOTIF: active site MOTIF: cysteine residue

References

  1. Kosik KS, Shimura H. Phosphorylated tau and the neurodegenerative foldopathies. Biochim Biophys Acta. 2005 Jan 3;1739(2-3):298-310. Epub 2004 Nov 26. Review. PMID: 15615647
  2. Pickart CM. Ubiquitin in chains. Trends Biochem Sci. 2000 Nov;25(11):544-8. Review. PMID: 11084366
  3. UniProt :accession P15374
  4. UniProt :accession P40818
  5. Brooks M FDA Clears First Blood Test to Aid in Concussion Diagnosis. Medscape - Feb 14, 2018. https://www.medscape.com/viewarticle/892683 - FDA News Release. Feb 14, 2018 FDA authorizes marketing of first blood test to aid in the evaluation of concussion in adults. https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm596531.htm