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Deficiency of IL-36R antagonist

Disease ID:1242
Name:Deficiency of IL-36R antagonist
Associated with:0 target
1 immuno-relevant ligand
The phenotype of this extremely rare autosomal recessive genetic abnormality covers a range of pustular skin syndromes that are variants of generalized pustular psoriasis (GPP). GPP is an acute, episodic, and potentially life-threatening form of psoriasis that presents as repeated episodes of cutaneous pustulosis and plaque development over a wide area of the body.
Database Links
OMIM: 614204
Orphanet: ORPHA404546


No target related data available for Deficiency of IL-36R antagonist


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Ligand References Clinical and Disease comments
Immuno Disease Comments: MAB92 is an experimental antibody with activity against DITRA type cutaneous pustulitis.
Bioactivity Comments: MAB92 blocks pro-inflammatory IL-36-induced actions such as NFκB activation and IL-8 production in primary human dermal fibroblasts and human keratinocytes in vitro [1]. | View biological activity


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1. Ganesan R, Raymond EL, Mennerich D, Woska Jr JR, Caviness G, Grimaldi C, Ahlberg J, Perez R, Roberts S, Yang D et al.. (2017) Generation and functional characterization of anti-human and anti-mouse IL-36R antagonist monoclonal antibodies. MAbs, 9 (7): 1143-1154. [PMID:28726542]