IKZF3

Zinc finger protein Aiolos also known as Ikaros family zinc finger protein 3 is a protein that in humans is encoded by the IKZF3 gene.

IKZF3
Identifiers
Aliases	IKZF3, AIO, AIOLOS, ZNFN1A3, IKAROS family zinc finger 3, IMD84
External IDs	OMIM: 606221; MGI: 1342542; HomoloGene: 8269; GeneCards: IKZF3; OMA:IKZF3 - orthologs
Gene location (Human) Chr. Chromosome 17 (human) Band 17q12-q21.1 Start 39,757,718 bp End 39,864,312 bp
Gene location (Mouse) Chr. Chromosome 11 (mouse) Band 11|11 D Start 98,355,728 bp End 98,436,857 bp
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in granulocyte lymph node epithelium of nasopharynx bone marrow cells thymus spleen blood tonsil superficial temporal artery appendix Top expressed in mesenteric lymph nodes thymus spleen blood lumbar subsegment of spinal cord female urethra subcutaneous adipose tissue submandibular gland granulocyte transitional epithelium of urinary bladder More reference expression data BioGPS More reference expression data
Gene ontology Molecular function sequence-specific DNA binding DNA binding RNA polymerase II transcription regulatory region sequence-specific DNA binding protein homodimerization activity DNA-binding transcription factor activity DNA-binding transcription activator activity, RNA polymerase II-specific metal ion binding protein binding protein heterodimerization activity nucleic acid binding identical protein binding DNA-binding transcription factor activity, RNA polymerase II-specific Cellular component cytoplasm plasma membrane nucleus nucleoplasm cytosol Biological process regulation of apoptotic process regulation of transcription, DNA-templated regulation of transcription by RNA polymerase II transcription by RNA polymerase II transcription, DNA-templated B cell activation regulation of B cell proliferation regulation of lymphocyte differentiation mesoderm development regulation of B cell differentiation positive regulation of transcription by RNA polymerase II response to bacterium Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 22806 22780 Ensembl ENSG00000161405 ENSMUSG00000018168 UniProt Q9UKT9 O08900 RefSeq (mRNA) NM_183232 NM_001257408 NM_001257409 NM_001257410 NM_001257411 NM_001257412 NM_001257413 NM_001257414 NM_001284514 NM_001284515 NM_001284516 NM_012481 NM_183228 NM_183229 NM_183230 NM_183231 NM_011771 RefSeq (protein) NP_001244337 NP_001244338 NP_001244339 NP_001244340 NP_001244341 NP_001244342 NP_001244343 NP_001271443 NP_001271444 NP_001271445 NP_036613 NP_899051 NP_899052 NP_899053 NP_899054 NP_899055 NP_035901 Location (UCSC) Chr 17: 39.76 – 39.86 Mb Chr 11: 98.36 – 98.44 Mb PubMed search
Wikidata
View/Edit Human View/Edit Mouse

Function

This gene encodes a member of the Ikaros family of zinc-finger proteins. Three members of this protein family (Ikaros, Aiolos and Helios) are hematopoietic-specific transcription factors involved in the regulation of lymphocyte development. This gene product is a transcription factor that is important in the regulation of B lymphocyte proliferation and differentiation. Both Ikaros and Aiolos can participate in chromatin remodeling. Regulation of gene expression in B lymphocytes by Aiolos is complex as it appears to require the sequential formation of Ikaros homodimers, Ikaros/Aiolos heterodimers, and Aiolos homodimers. At least six alternative transcripts encoding different isoforms have been described.

Interactions

IKZF3 has been shown to interact with BCL2-like 1 and HRAS.

Further reading

• Sun J, Matthias G, Mihatsch MJ, Georgopoulos K, Matthias P (February 2003). "Lack of the transcriptional coactivator OBF-1 prevents the development of systemic lupus erythematosus-like phenotypes in Aiolos mutant mice". Journal of Immunology. 170 (4): 1699–706. doi:10.4049/jimmunol.170.4.1699. PMID 12574333. S2CID 31136931.
• Schmitt C, Tonnelle C, Dalloul A, Chabannon C, Debré P, Rebollo A (June 2002). "Aiolos and Ikaros: regulators of lymphocyte development, homeostasis and lymphoproliferation". Apoptosis. 7 (3): 277–84. doi:10.1023/A:1015372322419. PMID 11997672. S2CID 1318582.
• Wang JH, Avitahl N, Cariappa A, Friedrich C, Ikeda T, Renold A, Andrikopoulos K, Liang L, Pillai S, Morgan BA, Georgopoulos K (October 1998). "Aiolos regulates B cell activation and maturation to effector state". Immunity. 9 (4): 543–53. doi:10.1016/S1074-7613(00)80637-8. PMID 9806640.
• Kim J, Sif S, Jones B, Jackson A, Koipally J, Heller E, Winandy S, Viel A, Sawyer A, Ikeda T, Kingston R, Georgopoulos K (March 1999). "Ikaros DNA-binding proteins direct formation of chromatin remodeling complexes in lymphocytes". Immunity. 10 (3): 345–55. doi:10.1016/S1074-7613(00)80034-5. PMID 10204490.
• Koipally J, Renold A, Kim J, Georgopoulos K (June 1999). "Repression by Ikaros and Aiolos is mediated through histone deacetylase complexes". The EMBO Journal. 18 (11): 3090–100. doi:10.1093/emboj/18.11.3090. PMC 1171390. PMID 10357820.
• Romero F, Martínez-A C, Camonis J, Rebollo A (June 1999). "Aiolos transcription factor controls cell death in T cells by regulating Bcl-2 expression and its cellular localization". The EMBO Journal. 18 (12): 3419–30. doi:10.1093/emboj/18.12.3419. PMC 1171421. PMID 10369681.
• Koipally J, Georgopoulos K (June 2000). "Ikaros interactions with CtBP reveal a repression mechanism that is independent of histone deacetylase activity". The Journal of Biological Chemistry. 275 (26): 19594–602. doi:10.1074/jbc.M000254200. PMID 10766745.
• Perdomo J, Holmes M, Chong B, Crossley M (December 2000). "Eos and pegasus, two members of the Ikaros family of proteins with distinct DNA binding activities". The Journal of Biological Chemistry. 275 (49): 38347–54. doi:10.1074/jbc.M005457200. PMID 10978333.
• Rebollo A, Ayllón V, Fleischer A, Martínez CA, Zaballos A (December 2001). "The association of Aiolos transcription factor and Bcl-xL is involved in the control of apoptosis". Journal of Immunology. 167 (11): 6366–73. doi:10.4049/jimmunol.167.11.6366. PMID 11714801.
• Liippo J, Nera KP, Veistinen E, Lähdesmäki A, Postila V, Kimby E, Riikonen P, Hammarström L, Pelkonen J, Lassila O (December 2001). "Both normal and leukemic B lymphocytes express multiple isoforms of the human Aiolos gene". European Journal of Immunology. 31 (12): 3469–74. doi:10.1002/1521-4141(200112)31:12<3469::AID-IMMU3469>3.0.CO;2-G. PMID 11745366. S2CID 44722991.
• Nakase K, Ishimaru F, Fujii K, Tabayashi T, Kozuka T, Sezaki N, Matsuo Y, Harada M (April 2002). "Overexpression of novel short isoforms of Helios in a patient with T-cell acute lymphoblastic leukemia". Experimental Hematology. 30 (4): 313–7. doi:10.1016/S0301-472X(01)00796-2. PMID 11937265.
• Koipally J, Georgopoulos K (August 2002). "A molecular dissection of the repression circuitry of Ikaros". The Journal of Biological Chemistry. 277 (31): 27697–705. doi:10.1074/jbc.M201694200. PMID 12015313.
• Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M (October 2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature. 437 (7062): 1173–8. Bibcode:2005Natur.437.1173R. doi:10.1038/nature04209. PMID 16189514. S2CID 4427026.
• Antica M, Dubravcic K, Weber I, Raic L, Labar B, Batinic D (February 2007). "A search for a mutation of the Aiolos phosphorylation domain in lymphocytes from patients with leukemia". Haematologica. 92 (2): 260–1. doi:10.3324/haematol.10753. PMID 17296582.
• Ghadiri A, Duhamel M, Fleischer A, Reimann A, Dessauge F, Rebollo A (April 2007). "Critical function of Ikaros in controlling Aiolos gene expression". FEBS Letters. 581 (8): 1605–16. doi:10.1016/j.febslet.2007.03.025. PMID 17383641. S2CID 10676033.
• Caballero R, Setien F, Lopez-Serra L, Boix-Chornet M, Fraga MF, Ropero S, Megias D, Alaminos M, Sanchez-Tapia EM, Montoya MC, Esteller M, Gonzalez-Sarmiento R, Ballestar E (August 2007). "Combinatorial effects of splice variants modulate function of Aiolos". Journal of Cell Science. 120 (Pt 15): 2619–30. doi:10.1242/jcs.007344. hdl:10668/612. PMID 17646674.

External links

• IKZF3+protein,+human at the U.S. National Library of Medicine Medical Subject Headings (MeSH)

This article incorporates text from the United States National Library of Medicine, which is in the public domain.