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Disease-causing mutations in the XIAP BIR2 domain impair NOD2-dependent immune signalling.

Abstract:

X-linked Inhibitor of Apoptosis (XIAP) is an essential ubiquitin ligase for pro-inflammatory signalling downstream of the nucleotide-binding oligomerization domain containing (NOD)-1 and -2 pattern recognition receptors. Mutations in XIAP cause X-linked lymphoproliferative syndrome type-2 (XLP2), an immunodeficiency associated with a potentially fatal deregulation of the immune system, whose aetiology is not well understood. Here, we identify the XIAP baculovirus IAP repeat (BIR)2 domain as a...

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Publisher copy:
10.1002/emmm.201303090

Authors


Damgaard, RB More by this author
Speckmann, C More by this author
zur Stadt, U More by this author
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Journal:
EMBO molecular medicine
Volume:
5
Issue:
8
Pages:
1278-1295
Publication date:
2013-08-05
DOI:
EISSN:
1757-4684
ISSN:
1757-4676
URN:
uuid:75ec9946-530e-4689-bb0d-8b63360f2028
Source identifiers:
423552
Local pid:
pubs:423552

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