IL31RA: Associates with OSMR to form the interleukin-31 receptor which activates STAT3 and to a lower extent STAT1 and STAT5. May function in skin immunity. Defects in IL31RA are the cause of amyloidosis primary localized cutaneous type 2 (PLCA2). PLCA2 is primary amyloidosis characterized by localized cutaneous amyloid deposition. This condition usually presents with itching (especially on the lower legs) and visible changes of skin hyperpigmentation and thickening that may be exacerbated by chronic scratching and rubbing. Primary localized cutaneous amyloidosis is often divided into macular and lichen subtypes although many affected individuals often show both variants coexisting. Lichen amyloidosis characteristically presents as a pruritic eruption of grouped hyperkeratotic papules with a predilection for the shins, calves, ankles and dorsa of feet and thighs. Papules may coalesce to form hyperkeratotic plaques that can resemble lichen planus, lichen simplex or nodular prurigo. Macular amyloidosis is characterized by small pigmented macules that may merge to produce macular hyperpigmentation, sometimes with a reticulate or rippled pattern. In macular and lichen amyloidosis, amyloid is deposited in the papillary dermis in association with grouped colloid bodies, thought to represent degenerate basal keratinocytes. The amyloid deposits probably reflect a combination of degenerate keratin filaments, serum amyloid P component, and deposition of immunoglobulins. Belongs to the type I cytokine receptor family. Type 2 subfamily. 12 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, integral; Receptor, cytokine
Chromosomal Location of Human Ortholog: 5q11.2
Cellular Component: axon; cell junction; external side of plasma membrane; integral component of membrane; intracellular; plasma membrane; presynaptic membrane
Molecular Function: cytokine binding; hematopoietin/interferon-class (D200-domain) cytokine receptor activity; protein kinase binding; transcription coactivator activity
Biological Process: acute inflammatory response to antigenic stimulus; cytokine-mediated signaling pathway; defense response; glandular epithelial cell differentiation; homeostatic process; JAK-STAT cascade; macrophage differentiation; MAPK cascade; monocyte differentiation; negative regulation of apoptosis; negative regulation of macrophage activation; positive regulation of cell proliferation; positive regulation of transcription, DNA-templated; positive regulation of tyrosine phosphorylation of STAT protein; transmembrane receptor protein tyrosine kinase signaling pathway
Disease: Amyloidosis, Primary Localized Cutaneous, 2
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