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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)

Function: - deubiquitinase involved both in the processing of ubiquitin precursors & of ubiquitinated proteins - component of TGF-beta/BMP signaling cascade - regulates chromosome alignment & segregation in mitosis by regulating the localization of BIRC5/survivin to mitotic centromeres - specifically hydrolyzes both Lys-29- & Lys-33-linked polyubiquitins chains - specifically deubiquitinates monoubiquitinated SMAD4, opposing the activity of E3 ubiquitin-protein ligase TRIM33 - thiol-dependent hydrolysis of ester, thioester, amide, peptide & isopeptide bonds formed by the C-terminal Gly of ubiquitin - interacts with SMAD4, MARK4, NUAK1 & BIRC5/survivin Structure: belongs to the peptidase C19 family Compartment: cytoplasm Alternative splicing: named isoforms=2 Expression: - widely expressed in embryonic & adult tissues - escapes X-inactivation

Related

ubiquitin (UBCEP2, UBB, UBC)

General

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

Properties

SIZE: entity length = 2570 aa MW = 292 kD COMPARTMENT: cytoplasm MOTIF: Thr phosphorylation site {T590} cysteine residue {C1566} Ser phosphorylation site {S1600} Tyr phosphorylation site {Y1815} histidine residue {H1879} Ser phosphorylation site {S2443} Tyr phosphorylation site {Y2556} Ser phosphorylation site {S2563}

Database Correlations

OMIM 300072 UniProt Q93008 Pfam PF00443 Entrez Gene 8239 Kegg hsa:8239 ENZYME 3.4.19.12

References

  1. UniProt :accession Q93008
  2. OMIM :accession 300072