OTULIN deficiency causes auto-inflammatory syndrome.
Ubiquitin chains assembled via the N-terminal methionine (Met1 or linear ubiquitin), conjugated by the linear ubiquitin chain assembly complex (LUBAC), participate in NF-κΒ-dependent inflammatory signaling and immune responses. A recent report in Cell finds that OTULIN, a deubiquitinase that selectively cleaves Met1-linked ubiquitin chains, is essential for restraining inflammation in vivo.
Ludwig Institute for Cancer Research, Nuffield Department of Medicine, University of Oxford, Old Road Campus Research Building, Oxford OX3 7DQ, UK.
Berthe Katrine
BK
Fiil
Ludwig Institute for Cancer Research, Nuffield Department of Medicine, University of Oxford, Old Road Campus Research Building, Oxford OX3 7DQ, UK.
Mads
M
Gyrd-Hansen
D010450:Endopeptidases; D007113:Immunity, Innate; D008214:Lymphocytes; D018377:Neurotransmitter Agents; D025801:Ubiquitin
D018377:Neurotransmitter Agents; D025801:Ubiquitin; D010450:Endopeptidases
EMBO J. 2012 Oct 3;31(19):3856-70
23032187
Mol Cell. 2014 May 8;54(3):335-48
24726323
Genes Cells. 2014 Mar;19(3):254-72
24461064
Cell Rep. 2016 Mar 29;14 (12 ):2846-58
26997266
Immunol Rev. 2015 Jul;266(1):190-207
26085216
Cell Rep. 2015 Dec 15;13(10 ):2258-72
26670046
Proc Natl Acad Sci U S A. 2016 Sep 6;113(36):10127-32
27559085
EMBO J. 2012 Oct 3;31(19):3845-55
23032186
Nature. 2013 Jun 20;498(7454):318-24
23708998
Cell. 2016 Aug 25;166(5):1215-1230.e20
27523608
Cell. 2013 Jun 6;153(6):1312-26
23746843
Mol Cell. 2013 Jun 27;50(6):818-30
23806334