IL-36 receptor antagonist is also known as IL-36RA or IL36RN
, the latter being the gene symbol. It acts by antagonising the binding of IL-36α, β and γ to interleukin 1 receptor like 2 (IL1RL2
) which is the ligand binding component of the functional IL-36 receptor.
Abnormailites in the IL36RN
gene that produce mutated IL-36RA protein structures with compromised function have been identified as a cause of unregulated inflammatory cytokine secretion which drives the repeated inflammatory episodes that characterise the life-threatening condition, generalized pustular psoriasis [4-5
]. Where abnormal IL-36RA underlies generalized pustular psoriasis the condition can be referred to as DITRA (deficiency of interleukin 36 antagonist). DITRA has been identified as the cause of other pustular phenotypes, that have emerged as variants of pustular psoriasis [1
]. An antibody that mimics IL-36RA's antagonist activity at the IL-36 receptor (MAB92) has been described [2
]. MAB92 inhibits proinflammatory cytokine production in primary human keratinocytes and dermal fibroblasts in vitro