IKZF3

IKZF3
Identifiers
Aliases	IKZF3, AIO, AIOLOS, ZNFN1A3, IKAROS family zinc finger 3
External IDs	MGI: 1342542 HomoloGene: 8269 GeneCards: IKZF3
Genetically Related Diseases
primary biliary cirrhosis[1]
Gene ontology Molecular function • sequence-specific DNA binding • DNA binding • RNA polymerase II regulatory region sequence-specific DNA binding • protein homodimerization activity • transcription factor activity, sequence-specific DNA binding • transcriptional activator activity, RNA polymerase II transcription regulatory region sequence-specific binding • metal ion binding • protein binding • protein heterodimerization activity • nucleic acid binding • transcription regulatory region DNA binding Cellular component • cytoplasm • plasma membrane • nucleus Biological process • regulation of apoptotic process • regulation of transcription, DNA-templated • regulation of transcription from RNA polymerase II promoter • transcription from RNA polymerase II promoter • 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 from RNA polymerase II promoter Sources:Amigo / QuickGO
RNA expression pattern
More reference expression data
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_001244337.1 NP_001244338.1 NP_001244339.1 NP_001244340.1 NP_001244341.1 NP_001244342.1 NP_001244343.1 NP_001271443.1 NP_001271444.1 NP_001271445.1 NP_036613.2 NP_899051.1 NP_899052.1 NP_899053.1 NP_899054.1 NP_899055.1	NP_035901.1
Location (UCSC)	Chr 17: 39.76 – 39.86 Mb	Chr 11: 98.46 – 98.55 Mb
PubMed search	[2]	[3]
Wikidata

View/Edit Human

View/Edit Mouse

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.^[4][5][6]

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.^[6]

Interactions

IKZF3 has been shown to interact with BCL2-like 1^[7] and HRAS.^[8]

References

1. "Diseases that are genetically associated with IKZF3 view/edit references on wikidata".
2. "Human PubMed Reference:".
3. "Mouse PubMed Reference:".
4. Morgan B, Sun L, Avitahl N, Andrikopoulos K, Ikeda T, Gonzales E, Wu P, Neben S, Georgopoulos K (Jun 1997). "Aiolos, a lymphoid restricted transcription factor that interacts with Ikaros to regulate lymphocyte differentiation". EMBO J. 16 (8): 2004–13. doi:10.1093/emboj/16.8.2004. PMC 1169803. PMID 9155026.
5. Hosokawa Y, Maeda Y, Takahashi E, Suzuki M, Seto M (Feb 2000). "Human aiolos, an ikaros-related zinc finger DNA binding protein: cDNA cloning, tissue expression pattern, and chromosomal mapping". Genomics. 61 (3): 326–9. doi:10.1006/geno.1999.5949. PMID 10552935.
6. "Entrez Gene: IKZF3 IKAROS family zinc finger 3 (Aiolos)".
7. 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". J. Immunol. 167 (11): 6366–73. doi:10.4049/jimmunol.167.11.6366. PMID 11714801.
8. 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". EMBO J. 18 (12): 3419–30. doi:10.1093/emboj/18.12.3419. PMC 1171421. PMID 10369681.

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". J. Immunol. 170 (4): 1699–706. doi:10.4049/jimmunol.170.4.1699. PMID 12574333.
• Schmitt C, Tonnelle C, Dalloul A, et al. (2003). "Aiolos and Ikaros: regulators of lymphocyte development, homeostasis and lymphoproliferation.". Apoptosis. 7 (3): 277–84. doi:10.1023/A:1015372322419. PMID 11997672.
• Wang JH, Avitahl N, Cariappa A, et al. (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, et al. (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 (1999). "Repression by Ikaros and Aiolos is mediated through histone deacetylase complexes.". EMBO J. 18 (11): 3090–100. doi:10.1093/emboj/18.11.3090. PMC 1171390. PMID 10357820.
• Romero F, Martínez-A C, Camonis J, Rebollo A (1999). "Aiolos transcription factor controls cell death in T cells by regulating Bcl-2 expression and its cellular localization.". EMBO J. 18 (12): 3419–30. doi:10.1093/emboj/18.12.3419. PMC 1171421. PMID 10369681.
• Koipally J, Georgopoulos K (2000). "Ikaros interactions with CtBP reveal a repression mechanism that is independent of histone deacetylase activity.". J. Biol. Chem. 275 (26): 19594–602. doi:10.1074/jbc.M000254200. PMID 10766745.
• Perdomo J, Holmes M, Chong B, Crossley M (2001). "Eos and pegasus, two members of the Ikaros family of proteins with distinct DNA binding activities.". J. Biol. Chem. 275 (49): 38347–54. doi:10.1074/jbc.M005457200. PMID 10978333.
• Rebollo A, Ayllón V, Fleischer A, et al. (2002). "The association of Aiolos transcription factor and Bcl-xL is involved in the control of apoptosis.". J. Immunol. 167 (11): 6366–73. doi:10.4049/jimmunol.167.11.6366. PMID 11714801.
• Liippo J, Nera KP, Veistinen E, et al. (2002). "Both normal and leukemic B lymphocytes express multiple isoforms of the human Aiolos gene.". Eur. J. Immunol. 31 (12): 3469–74. doi:10.1002/1521-4141(200112)31:12<3469::AID-IMMU3469>3.0.CO;2-G. PMID 11745366.
• Nakase K, Ishimaru F, Fujii K, et al. (2002). "Overexpression of novel short isoforms of Helios in a patient with T-cell acute lymphoblastic leukemia.". Exp. Hematol. 30 (4): 313–7. doi:10.1016/S0301-472X(01)00796-2. PMID 11937265.
• Koipally J, Georgopoulos K (2002). "A molecular dissection of the repression circuitry of Ikaros.". J. Biol. Chem. 277 (31): 27697–705. doi:10.1074/jbc.M201694200. PMID 12015313.
• Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
• Rual JF, Venkatesan K, Hao T, et al. (2005). "Towards a proteome-scale map of the human protein-protein interaction network.". Nature. 437 (7062): 1173–8. doi:10.1038/nature04209. PMID 16189514.
• Antica M, Dubravcic K, Weber I, et al. (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, et al. (2007). "Critical function of Ikaros in controlling Aiolos gene expression.". FEBS Lett. 581 (8): 1605–16. doi:10.1016/j.febslet.2007.03.025. PMID 17383641.
• Caballero R, Setien F, Lopez-Serra L, et al. (2007). "Combinatorial effects of splice variants modulate function of Aiolos.". J. Cell. Sci. 120 (Pt 15): 2619–30. doi:10.1242/jcs.007344. PMID 17646674.

External links

• IKZF3 protein, human at the US 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.