{"entryType":"UniProtKB reviewed (Swiss-Prot)","primaryAccession":"Q13422","secondaryAccessions":["A4D260","B4E0Z1","D3DVM5","O00598","Q53XL2","Q69BM4","Q8WVA3"],"uniProtkbId":"IKZF1_HUMAN","entryAudit":{"firstPublicDate":"1998-12-15","lastAnnotationUpdateDate":"2026-09-02","lastSequenceUpdateDate":"1996-11-01","entryVersion":225,"sequenceVersion":1},"annotationScore":5.0,"organism":{"scientificName":"Homo sapiens","commonName":"Human","taxonId":9606,"lineage":["Eukaryota","Metazoa","Chordata","Craniata","Vertebrata","Euteleostomi","Mammalia","Eutheria","Euarchontoglires","Primates","Haplorrhini","Catarrhini","Hominidae","Homo"]},"proteinExistence":"1: Evidence at protein level","proteinDescription":{"recommendedName":{"fullName":{"value":"DNA-binding protein Ikaros"}},"alternativeNames":[{"fullName":{"value":"Ikaros family zinc finger protein 1"}},{"fullName":{"value":"Lymphoid transcription factor LyF-1"}}]},"genes":[{"geneName":{"value":"IKZF1"},"synonyms":[{"value":"IK1"},{"value":"IKAROS"},{"value":"LYF1"},{"value":"ZNFN1A1"}]}],"comments":[{"texts":[{"evidences":[{"evidenceCode":"ECO:0000250","source":"UniProtKB","id":"Q03267"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10204490"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"17135265"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"17934067"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"19141594"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"41565821"}],"value":"Transcription regulator of hematopoietic cell differentiation (PubMed:17934067). Binds gamma-satellite DNA (PubMed:17135265, PubMed:19141594). Plays a role in the development of lymphocytes, B- and T-cells. Binds and activates the enhancer (delta-A element) of the CD3-delta gene. Repressor of the TDT (fikzfterminal deoxynucleotidyltransferase) gene during thymocyte differentiation. Regulates transcription through association with both HDAC-dependent and HDAC-independent complexes. Targets the 2 chromatin-remodeling complexes, NuRD and BAF (SWI/SNF), in a single complex (PYR complex), to the beta-globin locus in adult erythrocytes (PubMed:10204490). Increases normal apoptosis in adult erythroid cells (By similarity). Confers early temporal competence to retinal progenitor cells (RPCs) (By similarity). Function is isoform-specific and is modulated by dominant-negative inactive isoforms (PubMed:17135265, PubMed:17934067). Recognized by CRBN and is targeted for degradation by DCX (DDB1-CUL4-X-box) E3 protein ligase complex (PubMed:41565821)"}],"commentType":"FUNCTION"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000250","source":"UniProtKB","id":"Q03267"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10204490"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10978333"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"41565821"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"17135265"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"17934067"}],"value":"Heterodimer formed by the various isoforms; this modulates transcription regulator activity (PubMed:17135265, PubMed:17934067). Heterodimer with other IKAROS family members. Interacts with IKZF4 AND IKZF5 (PubMed:10978333). Component of the chromatin-remodeling NuRD repressor complex which includes at least HDAC1, HDAC2, RBBP4, RBBP7, IKZF1, MTA2, MBD2, MBD3, MTA1L1, CHD3 and CHD4. Interacts directly with the CHD4 component of the NuRD complex. Interacts directly with SMARCA4; the interaction associates IKFZ1 with the BAF complex (PubMed:10204490). Interacts with SUMO1; the interaction sumoylates IKAROS, promoted by PIAS2 and PIAS3. Interacts with PIAS2 (isoform alpha); the interaction promotes sumoylation and reduces transcription repression (By similarity). Interacts, to a lesser extent, with PIAS3 (By similarity). Interacts with PPP1CC; the interaction targets PPP1CC to pericentromeric heterochromatin, dephosphorylates IKAROS, stabilizes it and prevents it from degradation (By similarity). Interacts with IKZF3 (By similarity). Interacts with CRBN; the interaction is direct and requires the presence of thalidomide or its derivatives (PubMed:41565821)"}],"commentType":"SUBUNIT"},{"commentType":"INTERACTION","interactions":[{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q8WTP8","geneName":"AEN","intActId":"EBI-8637627"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q9NX04","geneName":"AIRIM","intActId":"EBI-8643161"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q96Q83","geneName":"ALKBH3","intActId":"EBI-6658697"},"numberOfExperiments":5,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q9BXS5","geneName":"AP1M1","intActId":"EBI-541426"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q9H6L4","geneName":"ARMC7","intActId":"EBI-742909"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q7L5A3","geneName":"ATOSB","intActId":"EBI-745689"},"numberOfExperiments":6,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q13895","geneName":"BYSL","intActId":"EBI-358049"},"numberOfExperiments":5,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q8IYL3","geneName":"C1orf174","intActId":"EBI-715898"},"numberOfExperiments":4,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q9H7E9","geneName":"C8orf33","intActId":"EBI-715389"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q9HC52","geneName":"CBX8","intActId":"EBI-712912"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q8IYX8-2","geneName":"CEP57L1","intActId":"EBI-10181988"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"P61024","geneName":"CKS1B","intActId":"EBI-456371"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q13363-2","geneName":"CTBP1","intActId":"EBI-10171858"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"P56545","geneName":"CTBP2","intActId":"EBI-741533"},"numberOfExperiments":7,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"P56545-3","geneName":"CTBP2","intActId":"EBI-10171902"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"O43602","geneName":"DCX","intActId":"EBI-8646694"},"numberOfExperiments":4,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"P26196","geneName":"DDX6","intActId":"EBI-351257"},"numberOfExperiments":4,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q92630","geneName":"DYRK2","intActId":"EBI-749432"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q3B820","geneName":"FAM161A","intActId":"EBI-719941"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q9Y247","geneName":"FAM50B","intActId":"EBI-742802"},"numberOfExperiments":4,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"P61328","geneName":"FGF12","intActId":"EBI-6657662"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"Q96NE9","geneName":"FRMD6","intActId":"EBI-741729"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q13422","intActId":"EBI-745305"},"interactantTwo":{"uniProtKBAccession":"O76003","geneName":"GLRX3","intActId":"EBI-374781"},"numberOfExper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