Q9UKT9 (IKZF3_HUMAN) Reviewed, UniProtKB/Swiss-Prot
Last modified
May 1, 2013.
Version 107.
History...
Names·Attributes·General annotation·Ontologies·Interactions·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order
Names·Attributes·General annotation·Ontologies·Interactions·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize orderNames and origin
| Protein names | Recommended name: Zinc finger protein Aiolos Alternative name(s): Ikaros family zinc finger protein 3 | ||||
| Gene names |
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| Organism | Homo sapiens (Human) [Reference proteome] | ||||
| Taxonomic identifier | 9606 [NCBI] | ||||
| Taxonomic lineage | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo![]() |
Protein attributes
| Sequence length | 509 AA. |
| Sequence status | Complete. |
| Protein existence | Evidence at protein level |
General annotation (Comments)
| Function | Transcription factor that plays an important role in the regulation of lymphocyte differentiation. Plays an essential role in regulation of B-cell differentiation, proliferation and maturation to an effector state. Involved in regulating BCL2 expression and controlling apoptosis in T-cells in an IL2-dependent manner. Ref.6 |
| Subunit structure | Homodimer, and heterodimer with other IKAROS family members. Interacts with IKZF4 AND IKZF5. Interacts with IKZF1. Interacts with HRAS. Interacts with FOXP3; this interaction may be required for silencing target genes and regulating the suppressive activity of FOXP3-positive regulatory T-cells (Treg). Interacts with BCL21L isoform Bcl-X(L); this interaction blocks the anti-apoptotic role of BCL21L. Associates with histone deacetylase complexes containing HDAC1, MTA2 and SIN3A. Ref.3 Ref.6 Ref.7 Ref.8 Ref.9 |
| Subcellular location | |
| Tissue specificity | Expressed most strongly in peripheral blood leukocytes, the spleen, and the thymus. Ref.3 |
| Induction | Up-regulated by TGFB1 and 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) in activated AHR T-cells. Ref.9 |
| Post-translational modification | Phosphorylation on tyrosine residues induced by IL2 is required for dissociation from HRAS and nuclear translocation of IKZF3 in T-cells. Phosphorylation on tyrosine residues induced by IL4 is required for dissociation from Bcl-X(L) in T-cells. |
| Sequence similarities | Belongs to the Ikaros C2H2-type zinc-finger protein family. Contains 6 C2H2-type zinc fingers. |
Ontologies
Binary interactions
With | Entry | #Exp. | IntAct | Notes |
|---|---|---|---|---|
| FOXP3 | Q9BZS1 | 2 | EBI-747204,EBI-983719 |
Alternative products
| This entry describes 15 isoforms produced by alternative splicing. [Align] [Select] | ||||||
| Isoform 1 (identifier: Q9UKT9-1) Also known as: Aio-1; This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. | ||||||
| Isoform 2 (identifier: Q9UKT9-2) Also known as: Aio-del4; The sequence of this isoform differs from the canonical sequence as follows: 142-197: Missing. | ||||||
| Isoform 3 (identifier: Q9UKT9-3) Also known as: Aio-del5; The sequence of this isoform differs from the canonical sequence as follows: 198-236: Missing. | ||||||
| Isoform 4 (identifier: Q9UKT9-4) Also known as: Aio-del6; The sequence of this isoform differs from the canonical sequence as follows: 237-275: Missing. | ||||||
| Isoform 5 (identifier: Q9UKT9-5) Also known as: Aio-del4,5; The sequence of this isoform differs from the canonical sequence as follows: 142-236: Missing. | ||||||
| Isoform 6 (identifier: Q9UKT9-6) Also known as: Aio-del5,6; The sequence of this isoform differs from the canonical sequence as follows: 198-275: Missing. | ||||||
| Isoform 7 (identifier: Q9UKT9-7) Also known as: Aio-del2; The sequence of this isoform differs from the canonical sequence as follows: 21-54: Missing. | ||||||
| Isoform 8 (identifier: Q9UKT9-8) Also known as: Aio-del2,5; The sequence of this isoform differs from the canonical sequence as follows: 21-54: Missing. 198-236: Missing. | ||||||
| Isoform 9 (identifier: Q9UKT9-9) Also known as: Aio-del3; The sequence of this isoform differs from the canonical sequence as follows: 55-141: Missing. | ||||||
| Isoform 10 (identifier: Q9UKT9-10) Also known as: Aio-del3,4; The sequence of this isoform differs from the canonical sequence as follows: 55-197: Missing. | ||||||
| Isoform 11 (identifier: Q9UKT9-11) Also known as: Aio-del3,4,5; The sequence of this isoform differs from the canonical sequence as follows: 55-236: Missing. | ||||||
| Isoform 12 (identifier: Q9UKT9-12) Also known as: Aio-del3,4,5,6; The sequence of this isoform differs from the canonical sequence as follows: 55-275: Missing. | ||||||
| Isoform 13 (identifier: Q9UKT9-13) Also known as: Aio-del4,5,6; The sequence of this isoform differs from the canonical sequence as follows: 142-275: Missing. | ||||||
| Isoform 14 (identifier: Q9UKT9-14) Also known as: Aio-1-5a; The sequence of this isoform differs from the canonical sequence as follows: 237-263: ASAEARHIKAEMGSERALVLDRLASNV → GTGWGWVELSHLGIRLQDLNVPWCRLH 264-509: Missing. | ||||||
| Isoform 15 (identifier: Q9UKT9-15) Also known as: Aio-del4-5a; The sequence of this isoform differs from the canonical sequence as follows: 142-197: Missing. 237-263: ASAEARHIKAEMGSERALVLDRLASNV → GTGWGWVELSHLGIRLQDLNVPWCRLH |
Sequence annotation (Features)
| Feature key | Position(s) | Length | Description | Graphical view | Feature identifier | ||||
Molecule processing | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Chain | 1 – 509 | 509 | Zinc finger protein Aiolos | PRO_0000047090 | |||||
Regions | |||||||||
| Zinc finger | 118 – 140 | 23 | C2H2-type 1 | ||||||
| Zinc finger | 146 – 168 | 23 | C2H2-type 2 | ||||||
| Zinc finger | 174 – 196 | 23 | C2H2-type 3 | ||||||
| Zinc finger | 202 – 224 | 23 | C2H2-type 4; atypical | ||||||
| Zinc finger | 452 – 474 | 23 | C2H2-type 5 | ||||||
| Zinc finger | 480 – 504 | 25 | C2H2-type 6; atypical | ||||||
Natural variations | |||||||||
| Alternative sequence | 21 – 54 | 34 | Missing in isoform 7 and isoform 8. | VSP_041274 | |||||
| Alternative sequence | 55 – 275 | 221 | Missing in isoform 12. | VSP_041275 | |||||
| Alternative sequence | 55 – 236 | 182 | Missing in isoform 11. | VSP_041276 | |||||
| Alternative sequence | 55 – 197 | 143 | Missing in isoform 10. | VSP_041277 | |||||
| Alternative sequence | 55 – 141 | 87 | Missing in isoform 9. | VSP_041278 | |||||
| Alternative sequence | 142 – 275 | 134 | Missing in isoform 13. | VSP_041279 | |||||
| Alternative sequence | 142 – 236 | 95 | Missing in isoform 5. | VSP_006841 | |||||
| Alternative sequence | 142 – 197 | 56 | Missing in isoform 2 and isoform 15. | VSP_006840 | |||||
| Alternative sequence | 198 – 275 | 78 | Missing in isoform 6. | VSP_006843 | |||||
| Alternative sequence | 198 – 236 | 39 | Missing in isoform 3 and isoform 8. | VSP_006842 | |||||
| Alternative sequence | 237 – 275 | 39 | Missing in isoform 4. | VSP_006844 | |||||
| Alternative sequence | 237 – 263 | 27 | ASAEA…LASNV → GTGWGWVELSHLGIRLQDLN VPWCRLH in isoform 14 and isoform 15. | VSP_041280 | |||||
| Alternative sequence | 264 – 509 | 246 | Missing in isoform 14. | VSP_041281 | |||||
| Natural variant | 277 | 1 | E → Q Found in a renal cell carcinoma sample; somatic mutation. Ref.11 | VAR_064724 | |||||
Experimental info | |||||||||
| Sequence conflict | 321 | 1 | R → C in AAF13493. Ref.1 | ||||||
| Sequence conflict | 321 | 1 | R → C in CAC80427. Ref.2 | ||||||
| Sequence conflict | 321 | 1 | R → C in CAC80428. Ref.2 | ||||||
| Sequence conflict | 321 | 1 | R → C in CAC80429. Ref.2 | ||||||
| Sequence conflict | 321 | 1 | R → C in CAC80430. Ref.2 | ||||||
| Sequence conflict | 321 | 1 | R → C in CAC80431. Ref.2 | ||||||
| Sequence conflict | 361 | 1 | K → R in AAF13493. Ref.1 | ||||||
| Sequence conflict | 361 | 1 | K → R in CAC80427. Ref.2 | ||||||
| Sequence conflict | 361 | 1 | K → R in CAC80428. Ref.2 | ||||||
| Sequence conflict | 361 | 1 | K → R in CAC80429. Ref.2 | ||||||
| Sequence conflict | 361 | 1 | K → R in CAC80430. Ref.2 | ||||||
| Sequence conflict | 361 | 1 | K → R in CAC80431. Ref.2 | ||||||
| Sequence conflict | 365 | 1 | H → L in AAF13493. Ref.1 | ||||||
| Sequence conflict | 365 | 1 | H → L in CAC80427. Ref.2 | ||||||
| Sequence conflict | 365 | 1 | H → L in CAC80428. Ref.2 | ||||||
| Sequence conflict | 365 | 1 | H → L in CAC80429. Ref.2 | ||||||
| Sequence conflict | 365 | 1 | H → L in CAC80430. Ref.2 | ||||||
| Sequence conflict | 365 | 1 | H → L in CAC80431. Ref.2 | ||||||
| Sequence conflict | 443 | 1 | N → D in AAF13493. Ref.1 | ||||||
| Sequence conflict | 443 | 1 | N → D in CAC80427. Ref.2 | ||||||
| Sequence conflict | 443 | 1 | N → D in CAC80428. Ref.2 | ||||||
| Sequence conflict | 443 | 1 | N → D in CAC80429. Ref.2 | ||||||
| Sequence conflict | 443 | 1 | N → D in CAC80430. Ref.2 | ||||||
| Sequence conflict | 443 | 1 | N → D in CAC80431. Ref.2 | ||||||
| Sequence conflict | 487 | 1 | Y → D in AAF13493. Ref.1 | ||||||
| Sequence conflict | 487 | 1 | Y → D in CAC80427. Ref.2 | ||||||
| Sequence conflict | 487 | 1 | Y → D in CAC80428. Ref.2 | ||||||
| Sequence conflict | 487 | 1 | Y → D in CAC80429. Ref.2 | ||||||
| Sequence conflict | 487 | 1 | Y → D in CAC80430. Ref.2 | ||||||
| Sequence conflict | 487 | 1 | Y → D in CAC80431. Ref.2 | ||||||
| Sequence conflict | 506 | 1 | A → S in AAF13493. Ref.1 | ||||||
| Sequence conflict | 506 | 1 | A → S in CAC80427. Ref.2 | ||||||
| Sequence conflict | 506 | 1 | A → S in CAC80428. Ref.2 | ||||||
| Sequence conflict | 506 | 1 | A → S in CAC80429. Ref.2 | ||||||
| Sequence conflict | 506 | 1 | A → S in CAC80430. Ref.2 | ||||||
| Sequence conflict | 506 | 1 | A → S in CAC80431. Ref.2 | ||||||
Sequences
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References
| « Hide 'large scale' references | |
| [1] | "Human aiolos, an ikaros-related zinc finger DNA binding protein: cDNA cloning, tissue expression pattern, and chromosomal mapping." Hosokawa Y., Maeda Y., Takahashi E., Suzuki M., Seto M. Genomics 61:326-329(1999) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1). Tissue: Lymphoma. |
| [2] | "Both normal and leukemic B lymphocytes express multiple isoforms of the human Aiolos gene." Liippo J.P., Nera K.P., Veistinen E., Lahdesmaki A., Postila V., Kimby E., Riikonen P., Hammarstrom L., Pelkonen J., Lassila O. Eur. J. Immunol. 31:3469-3474(2001) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS 2; 3; 4; 5 AND 6), ALTERNATIVE SPLICING. Tissue: Lymphoid tissue. |
| [3] | "Combinatorial effects of splice variants modulate function of Aiolos." Caballero R., Setien F., Lopez-Serra L., Boix-Chornet M., Fraga M.F., Ropero S., Megias D., Alaminos M., Sanchez-Tapia E.M., Montoya M.C., Esteller M., Gonzalez-Sarmiento R., Ballestar E. J. Cell Sci. 120:2619-2630(2007) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS 7; 8; 9; 10; 11; 12; 13; 14 AND 15), SUBUNIT, SUBCELLULAR LOCATION, TISSUE SPECIFICITY, ALTERNATIVE SPLICING. |
| [4] | Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. Venter J.C.Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. |
| [5] | "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1). Tissue: Lymph. |
| [6] | "Aiolos transcription factor controls cell death in T cells by regulating Bcl-2 expression and its cellular localization." Romero F., Martinez-A C., Camonis J., Rebollo A. EMBO J. 18:3419-3430(1999) [PubMed] [Europe PMC] [Abstract] Cited for: FUNCTION, INTERACTION WITH HRAS, SUBCELLULAR LOCATION, PHOSPHORYLATION. |
| [7] | "Eos and pegasus, two members of the Ikaros family of proteins with distinct DNA binding activities." Perdomo J., Holmes M., Chong B., Crossley M. J. Biol. Chem. 275:38347-38354(2000) [PubMed] [Europe PMC] [Abstract] Cited for: INTERACTION WITH IKZF4 AND IKZF5. |
| [8] | "The association of Aiolos transcription factor and Bcl-xL is involved in the control of apoptosis." Rebollo A., Ayllon V., Fleischer A., Martinez C.A., Zaballos A. J. Immunol. 167:6366-6373(2001) [PubMed] [Europe PMC] [Abstract] Cited for: INTERACTION WITH BCL21L, PHOSPHORYLATION. |
| [9] | "Activation of the aryl hydrocarbon receptor induces human type 1 regulatory T cell-like and Foxp3(+) regulatory T cells." Gandhi R., Kumar D., Burns E.J., Nadeau M., Dake B., Laroni A., Kozoriz D., Weiner H.L., Quintana F.J. Nat. Immunol. 11:846-853(2010) [PubMed] [Europe PMC] [Abstract] Cited for: INTERACTION WITH FOXP3, INDUCTION. |
| [10] | "Initial characterization of the human central proteome." Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J. BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract] Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]. |
| [11] | "Exome sequencing identifies frequent mutation of the SWI/SNF complex gene PBRM1 in renal carcinoma." Varela I., Tarpey P., Raine K., Huang D., Ong C.K., Stephens P., Davies H., Jones D., Lin M.L., Teague J., Bignell G., Butler A., Cho J., Dalgliesh G.L., Galappaththige D., Greenman C., Hardy C., Jia M. Futreal P.A.Nature 469:539-542(2011) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANT GLN-277. |
| + | Additional computationally mapped references. |
Cross-references
Entry information
| Entry name | IKZF3_HUMAN | ||||||||
| Accession | Primary (citable) accession number: Q9UKT9 Secondary accession number(s): Q69BL6 Q8WWR3 | ||||||||
| Entry history |
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| Entry status | Reviewed (UniProtKB/Swiss-Prot) | ||||||||
| Annotation program | Chordata Protein Annotation Program | ||||||||
| Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. | ||||||||
Relevant documents
| Human chromosome 17 Human chromosome 17: entries, gene names and cross-references to MIM |
| Human entries with polymorphisms or disease mutations List of human entries with polymorphisms or disease mutations |
| Human polymorphisms and disease mutations Index of human polymorphisms and disease mutations |
| MIM cross-references Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot |
| SIMILARITY comments Index of protein domains and families |

Clusters with
