IKZF1

Summary

Gene Symbol: IKZF1
Description: IKAROS family zinc finger 1
Alias: CVID13, Hs.54452, IK1, IKAROS, LYF1, LyF-1, PPP1R92, PRO0758, ZNFN1A1, DNA-binding protein Ikaros, CLL-associated antigen KW-6, ikaros family zinc finger protein 1, lymphoid transcription factor LyF-1, protein phosphatase 1, regulatory subunit 92, zinc finger protein, subfamily 1A, 1 (Ikaros)
Species: human
Products:     IKZF1

Top Publications

  1. Iacobucci I, Lonetti A, Messa F, Cilloni D, Arruga F, Ottaviani E, et al. Expression of spliced oncogenic Ikaros isoforms in Philadelphia-positive acute lymphoblastic leukemia patients treated with tyrosine kinase inhibitors: implications for a new mechanism of resistance. Blood. 2008;112:3847-55 pubmed publisher
    b>Ikaros plays an important role in the control of differentiation and proliferation of all lymphoid lineages...
  2. Kim J, Sif S, Jones B, Jackson A, Koipally J, Heller E, et al. Ikaros DNA-binding proteins direct formation of chromatin remodeling complexes in lymphocytes. Immunity. 1999;10:345-55 pubmed
    The Ikaros gene family encodes zinc finger DNA-binding proteins essential for lineage determination and control of proliferation in the lymphoid system...
  3. Evans T, Milne E, Anderson D, de Klerk N, Jamieson S, Talseth Palmer B, et al. Confirmation of childhood acute lymphoblastic leukemia variants, ARID5B and IKZF1, and interaction with parental environmental exposures. PLoS ONE. 2014;9:e110255 pubmed publisher
    Genome wide association studies (GWAS) have established association of ARID5B and IKZF1 variants with childhood acute lymphoblastic leukemia (ALL)...
  4. Ma H, Qazi S, Ozer Z, Zhang J, Ishkhanian R, Uckun F. Regulatory phosphorylation of Ikaros by Bruton's tyrosine kinase. PLoS ONE. 2013;8:e71302 pubmed publisher
    Diminished Ikaros function has been implicated in the pathogenesis of acute lymphoblastic leukemia (ALL), the most common form of childhood cancer...
  5. Linabery A, Blommer C, Spector L, Davies S, Robison L, Ross J. ARID5B and IKZF1 variants, selected demographic factors, and childhood acute lymphoblastic leukemia: a report from the Children's Oncology Group. Leuk Res. 2013;37:936-42 pubmed publisher
    Interactions between common germline variants in ARID5B and IKZF1 and other known childhood acute lymphoblastic leukemia (ALL) risk factors were queried using biospecimens and data from 770 ALL cases and 384 controls...
  6. Liu P, Lin Z, Qian S, Qiao C, Qiu H, Wu Y, et al. Expression of dominant-negative Ikaros isoforms and associated genetic alterations in Chinese adult patients with leukemia. Ann Hematol. 2012;91:1039-49 pubmed publisher
    Dominant-negative (DN) Ikaros isoforms, having important roles in pathogenesis of leukemia, are mainly studied in pediatric patients, but little is known about Chinese adult patients...
  7. Jager R, Gisslinger H, Passamonti F, Rumi E, Berg T, Gisslinger B, et al. Deletions of the transcription factor Ikaros in myeloproliferative neoplasms. Leukemia. 2010;24:1290-8 pubmed publisher
    ..We mapped the common deleted region to the IKZF1 gene, which encodes the transcription factor Ikaros. We further examined the frequency of IKZF1 deletions in a total of 29 post-MPN leukemia and 526 MPN patients ..
  8. Dijon M, Bardin F, Murati A, Batoz M, Chabannon C, Tonnelle C. The role of Ikaros in human erythroid differentiation. Blood. 2008;111:1138-46 pubmed
    b>Ikaros--a factor that positively or negatively controls gene transcription--is active in murine adult erythroid cells, and involved in fetal to adult globin switching. Mice with Ikaros mutations have defects in erythropoiesis and anemia...
  9. Zhou F, Mei H, Jin R, Li X, Chen X. Expression of Ikaros isoform 6 in chinese children with acute lymphoblastic leukemia. J Pediatr Hematol Oncol. 2011;33:429-32 pubmed publisher
    b>Ikaros is a zinc-finger transcription factor that plays an important role in the differentiation and proliferation of lymphocytes...

More Information

Publications73

  1. Yang L, Luo Y, Wei J. Integrative genomic analyses on Ikaros and its expression related to solid cancer prognosis. Oncol Rep. 2010;24:571-7 pubmed
    b>Ikaros is a member of the Kruppel family of zinc finger DNA-binding proteins...
  2. Harvey R, Mullighan C, Chen I, Wharton W, Mikhail F, Carroll A, et al. Rearrangement of CRLF2 is associated with mutation of JAK kinases, alteration of IKZF1, Hispanic/Latino ethnicity, and a poor outcome in pediatric B-progenitor acute lymphoblastic leukemia. Blood. 2010;115:5312-21 pubmed publisher
    ..rearrangements were significantly associated with activating mutations of JAK1 or JAK2, deletion or mutation of IKZF1, and Hispanic/Latino ethnicity (Fisher exact test, P < .001 for each)...
  3. Han J, Zheng H, Cui Y, Sun L, Ye D, Hu Z, et al. Genome-wide association study in a Chinese Han population identifies nine new susceptibility loci for systemic lupus erythematosus. Nat Genet. 2009;41:1234-7 pubmed publisher
    ..We identified nine new susceptibility loci (ETS1, IKZF1, RASGRP3, SLC15A4, TNIP1, 7q11.23, 10q11.22, 11q23.3 and 16p11.2; 1.77 x 10(-25) < or = P(combined) < or = 2...
  4. Treviño L, Yang W, French D, Hunger S, Carroll W, Devidas M, et al. Germline genomic variants associated with childhood acute lymphoblastic leukemia. Nat Genet. 2009;41:1001-5 pubmed publisher
    ..86, respectively) and were associated with methotrexate accumulation and gene expression pattern in leukemic lymphoblasts. We conclude that germline variants affect susceptibility to, and characteristics of, specific ALL subtypes. ..
  5. Georgopoulos K, Moore D, Derfler B. Ikaros, an early lymphoid-specific transcription factor and a putative mediator for T cell commitment. Science. 1992;258:808-12 pubmed
    ..control differentiation into the T cell lineage, a complementary DNA was isolated encoding a zinc finger protein (Ikaros) related to the Drosophila gap protein Hunchback...
  6. Sun L, Heerema N, Crotty L, Wu X, Navara C, Vassilev A, et al. Expression of dominant-negative and mutant isoforms of the antileukemic transcription factor Ikaros in infant acute lymphoblastic leukemia. Proc Natl Acad Sci U S A. 1999;96:680-5 pubmed
    b>Ikaros, a zinc finger-containing DNA-binding protein, is required for normal lymphocyte development, and germline mutant mice that express only non-DNA binding dominant-negative "leukemogenic" Ikaros isoforms lacking critical N-..
  7. Uckun F, Ma H, Zhang J, Ozer Z, Dovat S, Mao C, et al. Serine phosphorylation by SYK is critical for nuclear localization and transcription factor function of Ikaros. Proc Natl Acad Sci U S A. 2012;109:18072-7 pubmed publisher
    b>Ikaros is a zinc finger-containing DNA-binding protein that plays a pivotal role in immune homeostasis through transcriptional regulation of the earliest stages of lymphocyte ontogeny and differentiation...
  8. Chen I, Harvey R, Mullighan C, Gastier Foster J, Wharton W, Kang H, et al. Outcome modeling with CRLF2, IKZF1, JAK, and minimal residual disease in pediatric acute lymphoblastic leukemia: a Children's Oncology Group study. Blood. 2012;119:3512-22 pubmed publisher
    ..P2RY8-CRLF2, CRLF2 F232C), deletion/mutation in genes frequently associated with high CRLF2 expression (IKZF1, JAK, IL7R), and minimal residual disease (MRD) in 1061 pediatric ALL patients (499 HR and 562 SR) on COG Trials ..
  9. Ezzat S, Yu S, Asa S. Ikaros isoforms in human pituitary tumors: distinct localization, histone acetylation, and activation of the 5' fibroblast growth factor receptor-4 promoter. Am J Pathol. 2003;163:1177-84 pubmed
    ..We have shown that FGFR4 is a target for Ikaros, a zinc finger-containing transcription factor that localizes to heterochromatin regions and participates in ..
  10. Mullighan C, Miller C, Radtke I, Phillips L, Dalton J, Ma J, et al. BCR-ABL1 lymphoblastic leukaemia is characterized by the deletion of Ikaros. Nature. 2008;453:110-4 pubmed publisher
    ..IKZF1 (encoding the transcription factor Ikaros) was deleted in 83.7% of BCR-ABL1 ALL, but not in chronic-phase CML...
  11. Waanders E, van der Velden V, van der Schoot C, van Leeuwen F, van Reijmersdal S, de Haas V, et al. Integrated use of minimal residual disease classification and IKZF1 alteration status accurately predicts 79% of relapses in pediatric acute lymphoblastic leukemia. Leukemia. 2011;25:254-8 pubmed publisher
    ..We analyzed MRD in 131 uniformly treated precursor-B-ALL patients and evaluated whether combined MRD and IKZF1 (Ikaros zinc finger-1) alteration status can improve risk stratification...
  12. Mullighan C, Su X, Zhang J, Radtke I, Phillips L, Miller C, et al. Deletion of IKZF1 and prognosis in acute lymphoblastic leukemia. N Engl J Med. 2009;360:470-80 pubmed publisher
    ..predictor was strongly associated with alteration of IKZF1, a gene that encodes the lymphoid transcription factor IKAROS. The gene-expression signature of the group of patients with a poor outcome revealed increased expression of ..
  13. Popescu M, Gurel Z, Ronni T, Song C, Hung K, Payne K, et al. Ikaros stability and pericentromeric localization are regulated by protein phosphatase 1. J Biol Chem. 2009;284:13869-80 pubmed publisher
    b>Ikaros encodes a zinc finger protein that is involved in gene regulation and chromatin remodeling. The majority of Ikaros localizes at pericentromeric heterochromatin (PC-HC) where it regulates expression of target genes...
  14. Sun L, Goodman P, Wood C, Crotty M, Sensel M, Sather H, et al. Expression of aberrantly spliced oncogenic ikaros isoforms in childhood acute lymphoblastic leukemia. J Clin Oncol. 1999;17:3753-66 pubmed
    We sought to determine if molecular abnormalities involving the Ikaros gene could contribute to the development of acute lymphoblastic leukemia (ALL) in children...
  15. Koipally J, Georgopoulos K. Ikaros interactions with CtBP reveal a repression mechanism that is independent of histone deacetylase activity. J Biol Chem. 2000;275:19594-602 pubmed
    We have previously shown that Ikaros can repress transcription through the recruitment of histone deacetylase complexes...
  16. Migliorini G, Fiege B, Hosking F, Ma Y, Kumar R, Sherborne A, et al. Variation at 10p12.2 and 10p14 influences risk of childhood B-cell acute lymphoblastic leukemia and phenotype. Blood. 2013;122:3298-307 pubmed publisher
    ..0001). These findings provide further insights into the genetic and biological basis of inherited genetic susceptibility to BCP-ALL and the influence of constitutional genotype on disease development. ..
  17. Trageser D, Iacobucci I, Nahar R, Duy C, von Levetzow G, Klemm L, et al. Pre-B cell receptor-mediated cell cycle arrest in Philadelphia chromosome-positive acute lymphoblastic leukemia requires IKAROS function. J Exp Med. 2009;206:1739-53 pubmed publisher
    ..clinical outcome among all ALL subtypes and is defined by the oncogenic BCR-ABL1 kinase and deletions of the IKAROS gene in >80% of cases...
  18. Klein F, Feldhahn N, Herzog S, Sprangers M, Mooster J, Jumaa H, et al. BCR-ABL1 induces aberrant splicing of IKAROS and lineage infidelity in pre-B lymphoblastic leukemia cells. Oncogene. 2006;25:1118-24 pubmed
    ..Consistent with this, BCR-ABL1+ pre-B lymphoblastic leukemia cells exhibit defective expression of IKAROS, a transcription factor needed for early lymphoid lineage commitment...
  19. Wang Y, Chen J, Li J, Deng J, Rui Y, Lu Q, et al. Association of three polymorphisms in ARID5B, IKZF1 and CEBPE with the risk of childhood acute lymphoblastic leukemia in a Chinese population. Gene. 2013;524:203-7 pubmed publisher
    ..in children younger than 15 years old, have found evidence that single nucleotide polymorphisms (SNPs) in IKZF1 (7p12.2), ARID5B (10q21.2) and CEBPE (14q11.2) are strongly related to the risk of childhood ALL...
  20. Martinelli G, Iacobucci I, Storlazzi C, Vignetti M, Paoloni F, Cilloni D, et al. IKZF1 (Ikaros) deletions in BCR-ABL1-positive acute lymphoblastic leukemia are associated with short disease-free survival and high rate of cumulative incidence of relapse: a GIMEMA AL WP report. J Clin Oncol. 2009;27:5202-7 pubmed publisher
  21. O Neill D, Schoetz S, Lopez R, Castle M, Rabinowitz L, Shor E, et al. An ikaros-containing chromatin-remodeling complex in adult-type erythroid cells. Mol Cell Biol. 2000;20:7572-82 pubmed
    ..In addition, we show that the hematopoietic cell-restricted zinc finger protein Ikaros copurifies with PYR complex DNA-binding activity and that antibodies to Ikaros also supershift the complex...
  22. Koipally J, Georgopoulos K. A molecular dissection of the repression circuitry of Ikaros. J Biol Chem. 2002;277:27697-705 pubmed
    b>Ikaros is a key regulator of the hemo-lymphoid system in which it is presumed to function by both potentiating and repressing gene expression...
  23. Yang Y, Hung C, Chen J, Lin K, Jou S, Hsiao C, et al. IKZF1 deletions predict a poor prognosis in children with B-cell progenitor acute lymphoblastic leukemia: a multicenter analysis in Taiwan. Cancer Sci. 2011;102:1874-81 pubmed publisher
    ..Recent genome-wide analyses have identified that an alteration of IKZF1 is associated with very poor outcomes in B-cell progenitor ALL...
  24. Papaemmanuil E, Hosking F, Vijayakrishnan J, Price A, Olver B, Sheridan E, et al. Loci on 7p12.2, 10q21.2 and 14q11.2 are associated with risk of childhood acute lymphoblastic leukemia. Nat Genet. 2009;41:1006-10 pubmed publisher
    ..We identified risk loci for ALL at 7p12.2 (IKZF1, rs4132601, odds ratio (OR) = 1.69, P = 1.20 x 10(-19)), 10q21.2 (ARID5B, rs7089424, OR = 1.65, P = 6...
  25. Kano G, Morimoto A, Takanashi M, Hibi S, Sugimoto T, Inaba T, et al. Ikaros dominant negative isoform (Ik6) induces IL-3-independent survival of murine pro-B lymphocytes by activating JAK-STAT and up-regulating Bcl-xl levels. Leuk Lymphoma. 2008;49:965-73 pubmed publisher
    b>Ikaros is an essential regulator of lymphocyte differentiation. Mice transgenic for the Ikaros dominant negative (DN) mutation rapidly develop lymphoid malignancies...
  26. Kuiper R, Waanders E, van der Velden V, van Reijmersdal S, Venkatachalam R, Scheijen B, et al. IKZF1 deletions predict relapse in uniformly treated pediatric precursor B-ALL. Leukemia. 2010;24:1258-64 pubmed publisher
    ..In contrast, deletions and nonsense mutations in IKZF1 (IKAROS) were highly enriched and consistently preserved at the time of relapse...
  27. Payne K, Dovat S. Ikaros and tumor suppression in acute lymphoblastic leukemia. Crit Rev Oncog. 2011;16:3-12 pubmed
    The Ikzf1 gene encodes Ikaros-a DNA-binding zinc finger protein. Ikaros functions as a regulator of gene expression and chromatin remodeling...
  28. Vijayakrishnan J, Sherborne A, Sawangpanich R, Hongeng S, Houlston R, Pakakasama S. Variation at 7p12.2 and 10q21.2 influences childhood acute lymphoblastic leukemia risk in the Thai population and may contribute to racial differences in leukemia incidence. Leuk Lymphoma. 2010;51:1870-4 pubmed publisher
    ..g. IKZF1, rs4132601; risk allele frequency [RAF] ratio of 0.36 for Thai/Caucasian)...
  29. He L, Xu H, Gu Z, Liu C, Chen G, Wang Y, et al. Ikaros is degraded by proteasome-dependent mechanism in the early phase of apoptosis induction. Biochem Biophys Res Commun. 2011;406:430-4 pubmed publisher
    b>Ikaros is an important transcription factor involved in the development and differentiation of hematopoietic cells...
  30. Xu H, Yang W, Pérez Andreu V, Devidas M, Fan Y, Cheng C, et al. Novel susceptibility variants at 10p12.31-12.2 for childhood acute lymphoblastic leukemia in ethnically diverse populations. J Natl Cancer Inst. 2013;105:733-42 pubmed publisher
    ..001, .009, and .04, respectively). Association was also validated at four known ALL susceptibility loci: ARID5B, IKZF1, CEBPE, and CDKN2A/2B...
  31. Payne K, Nicolas J, Zhu J, Barsky L, Crooks G. Cutting edge: predominant expression of a novel Ikaros isoform in normal human hemopoiesis. J Immunol. 2001;167:1867-70 pubmed
    Murine studies implicate Ikaros proteins as regulators of hemopoiesis, particularly in the lymphoid lineages. High homology between murine and human Ikaros suggests that Ikaros expression in the two might be similar...
  32. Iacobucci I, Lonetti A, Cilloni D, Messa F, Ferrari A, Zuntini R, et al. Identification of different Ikaros cDNA transcripts in Philadelphia-positive adult acute lymphoblastic leukemia by a high-throughput capillary electrophoresis sizing method. Haematologica. 2008;93:1814-21 pubmed publisher
    b>Ikaros is the prototypic member of a Kruppel-like zinc finger transcription factor subfamily that is required for normal hematopoietic cell differentiation and proliferation, particularly in the lymphoid lineages...
  33. Prasad R, Hosking F, Vijayakrishnan J, Papaemmanuil E, Koehler R, Greaves M, et al. Verification of the susceptibility loci on 7p12.2, 10q21.2, and 14q11.2 in precursor B-cell acute lymphoblastic leukemia of childhood. Blood. 2010;115:1765-7 pubmed publisher
    Recent genome-wide association data have implicated genetic variation at 7p12.2 (IKZF1), 10q21.2 (ARIDB5), and 14q11.2 (CEBPE) in the etiology of B-cell childhood acute lymphoblastic leukemia (ALL)...
  34. Ellinghaus E, Stanulla M, Richter G, Ellinghaus D, te Kronnie G, Cario G, et al. Identification of germline susceptibility loci in ETV6-RUNX1-rearranged childhood acute lymphoblastic leukemia. Leukemia. 2012;26:902-9 pubmed publisher
    ..The identification of TP63 and PTPRJ as susceptibility genes emphasize the role of the TP53 gene family and the importance of proteins regulating cellular processes in connection with tumorigenesis. ..
  35. Dorman K, Shen Z, Yang C, Ezzat S, Asa S. CtBP1 interacts with Ikaros and modulates pituitary tumor cell survival and response to hypoxia. Mol Endocrinol. 2012;26:447-57 pubmed publisher
    ..CtBP interacts with Ikaros isoforms in GH4 and AtT20 pituitary tumor cells...
  36. Koipally J, Renold A, Kim J, Georgopoulos K. Repression by Ikaros and Aiolos is mediated through histone deacetylase complexes. EMBO J. 1999;18:3090-100 pubmed
    Here we show that the lymphoid lineage-determining factors Ikaros and Aiolos can function as strong transcriptional repressors...
  37. Tonnelle C, Dijon M, Moreau T, Garulli C, Bardin F, Chabannon C. Stage specific over-expression of the dominant negative Ikaros 6 reveals distinct role of Ikaros throughout human B-cell differentiation. Mol Immunol. 2009;46:1736-43 pubmed publisher
    b>Ikaros is a transcription factor that acts both as an activator and as an inhibitor of gene expression in several hematopoietic lineages...
  38. Yap W, Yeoh E, Tay A, Brenner S, Venkatesh B. STAT4 is a target of the hematopoietic zinc-finger transcription factor Ikaros in T cells. FEBS Lett. 2005;579:4470-8 pubmed
    ..of the 5' flanking regions of human, mouse and pufferfish (Fugu rubripes) Stat4 genes revealed a high frequency of Ikaros (Ik) binding elements in all three species...
  39. Iacobucci I, Storlazzi C, Cilloni D, Lonetti A, Ottaviani E, Soverini S, et al. Identification and molecular characterization of recurrent genomic deletions on 7p12 in the IKZF1 gene in a large cohort of BCR-ABL1-positive acute lymphoblastic leukemia patients: on behalf of Gruppo Italiano Malattie Ematologiche dell'Adulto Acute . Blood. 2009;114:2159-67 pubmed publisher
    ..somatic copy number alteration was a focal deletion on 7p12 of IKZF1, which encodes the transcription factor Ikaros and was identified in 80 (75%) of 106 patients...
  40. Payne K, Huang G, Sahakian E, Zhu J, Barteneva N, Barsky L, et al. Ikaros isoform x is selectively expressed in myeloid differentiation. J Immunol. 2003;170:3091-8 pubmed
    The Ikaros gene is alternately spliced to generate multiple DNA-binding and nonbinding isoforms that have been implicated as regulators of hematopoiesis, particularly in the lymphoid lineages...
  41. Iacobucci I, Iraci N, Messina M, Lonetti A, Chiaretti S, Valli E, et al. IKAROS deletions dictate a unique gene expression signature in patients with adult B-cell acute lymphoblastic leukemia. PLoS ONE. 2012;7:e40934 pubmed publisher
    Deletions of IKAROS (IKZF1) frequently occur in B-cell precursor acute lymphoblastic leukemia (B-ALL) but the mechanisms by which they influence pathogenesis are unclear...
  42. Nishii K, Katayama N, Miwa H, Shikami M, Usui E, Masuya M, et al. Non-DNA-binding Ikaros isoform gene expressed in adult B-precursor acute lymphoblastic leukemia. Leukemia. 2002;16:1285-92 pubmed
    b>Ikaros, a zinc finger transcription factor, is essential for lymphoid development. Mutant mice expressing dominant-negative Ikaros gene (Ikaros) isoforms develop an aggressive form of lymphoid malignancies...
  43. Schaefer J, Montufar Solis D, Klein J. A role for IL-10 in the transcriptional regulation of Roquin-1. Gene. 2014;549:134-40 pubmed publisher
    ..Luciferase reporter assays conducted using eight transcription factor plasmids (STAT1, STAT3, STAT5, GATA2, c-Rel, IKZF1, IKZF2, and IKZF3) demonstrated that STAT1, STAT3, GATA2, and c-Rel increased Rc3h1 promoter activity, whereas ..
  44. von Palffy S, Bulaeva E, Babovic S, Kannan N, Knapp D, Wei L, et al. Dominant-negative IKAROS enhances IL-3-stimulated signaling in wild-type but not BCR-ABL1(+) mouse BA/F3 cells. Exp Hematol. 2015;43:514-23.e1-2 pubmed publisher
    Inactivating mutations in IKZF1, the gene that encodes the transcription factor IKAROS, are recurrent in poor-prognosis human B-cell leukemias, in which these mutations co-exist with BCR-ABL1 or other genetic changes that activate similar ..
  45. Gao S, Geng C, Song T, Lin X, Liu J, Cai Z, et al. Activation of c-Abl Kinase Potentiates the Anti-myeloma Drug Lenalidomide by Promoting DDA1 Protein Recruitment to the CRL4 Ubiquitin Ligase. J Biol Chem. 2017;292:3683-3691 pubmed publisher
    ..or genetic ablation of the Abl-DDB1-DDA1 axis decreases the ubiquitination of CRL4 substrates, including IKZF1 and IKZF3, in lenalidomide-treated multiple myeloma cells...
  46. Dorsam G, Goetzl E. Vasoactive intestinal peptide receptor-1 (VPAC-1) is a novel gene target of the hemolymphopoietic transcription factor Ikaros. J Biol Chem. 2002;277:13488-93 pubmed
    ..The human VPAC-1 5'-flanking region (1.4 kb) contains four high affinity Ikaros (IK) consensus sequences...
  47. Goldman F, Gurel Z, Al Zubeidi D, Fried A, Icardi M, Song C, et al. Congenital pancytopenia and absence of B lymphocytes in a neonate with a mutation in the Ikaros gene. Pediatr Blood Cancer. 2012;58:591-7 pubmed publisher
    ..Due to the similarity of the patient's phenotype with the IKAROS knockout mouse, studies were performed on bone marrow and peripheral blood to assess a potential pathogenic role ..
  48. Matyskiela M, Zhang W, Man H, Muller G, Khambatta G, Baculi F, et al. A Cereblon Modulator (CC-220) with Improved Degradation of Ikaros and Aiolos. J Med Chem. 2018;61:535-542 pubmed publisher
    ..and pomalidomide bind to the protein cereblon, directing the CRL4-CRBN E3 ligase toward the transcription factors Ikaros and Aiolos to cause their ubiquitination and degradation...
  49. Li Y, Hu H, Tian J, Zhu M, O Neil R. Dynamic coupling between TRPV4 and Ca2+-activated SK1/3 and IK1 K+ channels plays a critical role in regulating the K+-secretory BK channel in kidney collecting duct cells. Am J Physiol Renal Physiol. 2017;312:F1081-F1089 pubmed publisher
    ..small-/intermediate-conductance Ca2+-activated K+ channels, SK1 (KCNN1), SK3 (KCNN3), and IK1 (KCNN4), with notably high Ca2+-binding affinities, that are expressed in CNT/CCD and may be regulated ..
  50. Boer J, van der Veer A, Rizopoulos D, Fiocco M, Sonneveld E, de Groot Kruseman H, et al. Prognostic value of rare IKZF1 deletion in childhood B-cell precursor acute lymphoblastic leukemia: an international collaborative study. Leukemia. 2016;30:32-8 pubmed publisher
    Deletions in IKZF1 are found in ~15% of children with B-cell precursor acute lymphoblastic leukemia (BCP-ALL)...
  51. Hannah J, Zhou P. Distinct and overlapping functions of the cullin E3 ligase scaffolding proteins CUL4A and CUL4B. Gene. 2015;573:33-45 pubmed publisher
    ..drugs are caused by their binding to the CRL4CRBN complex and re-directing the E3 ligase towards the Ikaros transcription factors IKZF1 and IKZF3...
  52. Oravecz A, Apostolov A, Polak K, Jost B, Le Gras S, Chan S, et al. Ikaros mediates gene silencing in T cells through Polycomb repressive complex 2. Nat Commun. 2015;6:8823 pubmed publisher
    ..How transcription factors influence these changes remains unclear. We show that the Ikaros transcription factor forms a complex with Polycomb repressive complex 2 (PRC2) in CD4(-)CD8(-) thymocytes and ..
  53. Churchman M, Mullighan C. Ikaros: Exploiting and targeting the hematopoietic stem cell niche in B-progenitor acute lymphoblastic leukemia. Exp Hematol. 2017;46:1-8 pubmed publisher
    Genetic alterations of IKZF1 encoding the lymphoid transcription factor IKAROS are a hallmark of high-risk B-progenitor acute lymphoblastic leukemia (ALL), such as BCR-ABL1-positive (Ph+) and Ph-like ALL, and are associated with poor ..
  54. Buitenkamp T, Pieters R, Zimmermann M, de Haas V, Richards S, Vora A, et al. BTG1 deletions do not predict outcome in Down syndrome acute lymphoblastic leukemia. Leukemia. 2013;27:251-2 pubmed publisher
  55. Gorniak P, Pastorczak A, Zalewska Szewczyk B, Lejman M, Trelinska J, Chmielewska M, et al. Polymorphism in IKZF1 gene affects age at onset of childhood acute lymphoblastic leukemia. Leuk Lymphoma. 2014;55:2174-8 pubmed publisher
    ..2 (IKZF1 gene) confers an increased risk of pediatric ALL, we investigated whether the risk allele at rs4132601 also ..
  56. Wojcik H, Griffiths E, Staggs S, Hagman J, Winandy S. Expression of a non-DNA-binding Ikaros isoform exclusively in B cells leads to autoimmunity but not leukemogenesis. Eur J Immunol. 2007;37:1022-32 pubmed
    b>Ikaros is a transcriptional regulator whose function is essential for B cell development. It is expressed in the hematopoietic stem cell (HSC) through the mature B cell stage...
  57. Yamashita Y, Shimada A, Yamada T, Yamaji K, Hori T, Tsurusawa M, et al. IKZF1 and CRLF2 gene alterations correlate with poor prognosis in Japanese BCR-ABL1-negative high-risk B-cell precursor acute lymphoblastic leukemia. Pediatr Blood Cancer. 2013;60:1587-92 pubmed publisher
    Genome-wide analysis studies have demonstrated that IKZF1, CRLF2, and JAK2 gene alterations correlate with poor prognosis in pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL)...
  58. Hsu L, Chokkalingam A, Briggs F, Walsh K, Crouse V, Fu C, et al. Association of genetic variation in IKZF1, ARID5B, and CEBPE and surrogates for early-life infections with the risk of acute lymphoblastic leukemia in Hispanic children. Cancer Causes Control. 2015;26:609-19 pubmed publisher
    Genome-wide association studies focusing on European-ancestry populations have identified ALL risk loci on IKZF1, ARID5B, and CEBPE...
  59. Diaz T, Rodriguez V, Lozano E, Mena M, Calderón M, Rosinol L, et al. The BET bromodomain inhibitor CPI203 improves lenalidomide and dexamethasone activity in in vitro and in vivo models of multiple myeloma by blockade of Ikaros and MYC signaling. Haematologica. 2017;102:1776-1784 pubmed publisher
    ..Clinical activity of lenalidomide relies on degradation of Ikaros and the consequent reduction in IRF4 expression, both required for myeloma cell survival and involved in the ..
  60. Goversen B, van der Heyden M, van Veen T, De Boer T. The immature electrophysiological phenotype of iPSC-CMs still hampers in vitro drug screening: Special focus on IK1. Pharmacol Ther. 2018;183:127-136 pubmed publisher
    ..We conclude that reduced densities of IK1 remain a major flaw in iPSC-CMs, which hampers their use for in vitro drug screening. ..
  61. Theocharides A, Dobson S, Laurenti E, Notta F, Voisin V, Cheng P, et al. Dominant-negative Ikaros cooperates with BCR-ABL1 to induce human acute myeloid leukemia in xenografts. Leukemia. 2015;29:177-87 pubmed publisher
    ..transplantation of cord blood transduced with vectors expressing BCR-ABL1 and a dominant-negative isoform of IKAROS, Ik6...
  62. Zhao Y, Huang H, Wei G. Novel agents and biomarkers for acute lymphoid leukemia. J Hematol Oncol. 2013;6:40 pubmed publisher
    ..leukemia (ALL) have been found to have prognostic impact, such as the lymphoid transcription factor gene IKZF1 alterations, which are associated with a high rate of leukemic relapse in B-ALL...
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