{"entity":{"id":"cd123","kind":"target","name":"CD123","aka":[],"tldr":"CD123 is the interleukin-3 receptor alpha chain, present on every blastic plasmacytoid dendritic cell neoplasm and on the blasts and leukaemia stem cells of 70 to 80% of acute myeloid leukaemias. Tagraxofusp and pivekimab sunirine target it in that rare neoplasm, and CAR-T cells against it in AML must spare the normal blood-forming progenitors that also carry it.","summary":"CD123 is the interleukin-3 receptor alpha chain (IL3RA), expressed on leukaemic stem cells and plasmacytoid dendritic cells. It is universally present in blastic plasmacytoid dendritic cell neoplasm (BPDCN) and found on blasts and leukaemic stem cells in roughly 70 to 80 percent of AML. Tagraxofusp, a CD123-directed cytotoxin, was the first approved therapy for BPDCN, and pivekimab sunirine (Decnupaz) followed with approval in 2026 for the same disease. CD123 is also a CAR-T target in AML, where the challenge is sparing normal haematopoietic progenitors that carry the antigen. Capillary leak syndrome with the fusion-toxin approach and durability of CAR-T responses remain open questions. The plain version: CD123 is an interleukin receptor over-abundant on a rare aggressive blood cancer and on leukaemia stem cells.","asOf":"2026-09-04","wikipedia":"https://en.wikipedia.org/wiki/Interleukin-3_receptor","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Interleukin-3_receptor"}],"tags":["adc-target"],"related":[],"cancers":["aml"],"sections":[],"technologies":[],"targets":[],"drugs":["pivekimab-sunirine","tagraxofusp"],"companies":["orum-therapeutics"],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"symbol":"IL3RA","role":[],"sources":[],"specificity":"lineage-antigen","distribution":"one-type","specificityNote":"Lineage antigen shared with normal granulocytes: HPA blood lineage lineage enriched at or above 25 nTPM, and 1 cell-killing or cell-finding medicine (Pivekimab sunirine) aim at it, so normal cells of the lineage are hit too. HPA IL3RA: RNA low tissue specificity; blood lineage lineage enriched (granulocytes 2,669 nTPM); no normal tissue stained high; highest cancer staining glioma (2 of 11 high). Distribution: 1 cancer family in the corpus carries a prevalence row, label threshold or catalogue link for it (Leukaemia); Open Targets associates it with 0 specific cancer types at or above 0.5. (Rule 5 of scripts/fetch-target-specificity.ts.)","specificitySources":[{"label":"Human Protein Atlas IL3RA tissue","url":"https://www.proteinatlas.org/ENSG00000185291-IL3RA/tissue","note":"RNA tissue and blood lineage specificity, normal tissue antibody staining (version 25.1, CC BY-SA 3.0)"},{"label":"Human Protein Atlas IL3RA pathology","url":"https://www.proteinatlas.org/ENSG00000185291-IL3RA/pathology","note":"patients per staining level per cancer type (version 25.1, CC BY-SA 3.0)"},{"label":"Open Targets ENSG00000185291 associations","url":"https://platform.opentargets.org/target/ENSG00000185291/associations","note":"cancer associations at or above 0.5 (CC0)"}],"hgnc":"HGNC:6012","ensembl":"ENSG00000185291","uniprot":"P26951","entrez":"3563","firstDescribed":1991,"firstDescribedBasis":"sequence","firstDescribedNote":"Earliest sequence paper UniProt cites for the protein: Kitamura et al, Cell, 1991, \"Expression cloning of the human IL-3 receptor cDNA reveals a shared beta subunit for the human IL-3 and GM-CSF receptors\".","firstDescribedSource":"https://pubmed.ncbi.nlm.nih.gov/1833064/","biology":"Expressed on leukaemic stem cells, plasmacytoid dendritic cells.","whereFound":["BPDCN","AML"],"targetClass":"surface-antigen","prevalence":[{"cancerId":"aml","pct":"70-80","measure":"Blasts and leukaemic stem cells","source":"https://en.wikipedia.org/wiki/Interleukin-3_receptor","note":"Universal in BPDCN"}]},"route":"/targets/cd123/","neighbours":{"cancer":[{"id":"aml","kind":"cancer","name":"Acute myeloid leukaemia","route":"/cancers/aml/"},{"id":"bpdcn","kind":"cancer","name":"Blastic plasmacytoid dendritic cell neoplasm (BPDCN)","route":"/cancers/bpdcn/"},{"id":"hairy-cell-leukemia","kind":"cancer","name":"Hairy cell leukaemia","route":"/cancers/hairy-cell-leukemia/"}],"drug":[{"id":"pivekimab-sunirine","kind":"drug","name":"Pivekimab sunirine","route":"/drugs/pivekimab-sunirine/"},{"id":"tagraxofusp","kind":"drug","name":"Tagraxofusp","route":"/drugs/tagraxofusp/"}],"company":[{"id":"orum-therapeutics","kind":"company","name":"Orum Therapeutics","route":"/companies/orum-therapeutics/"}],"technology":[{"id":"adc","kind":"technology","name":"Antibody-drug conjugate (ADC)","route":"/technologies/adc/"}]}}