IKZF3
Domain
C2H2-type 5 and C2H2-type 6 mediate homodimerization and heterodimerization.
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.
Involvement in disease
Immunodeficiency 84
IMD84
An autosomal recessive immunologic disorder characterized by recurrent sinopulmonary infections from childhood associated with low levels of B cells and impaired early B-cell development. There may also be variable T-cell abnormalities. Patients have increased susceptibility to infection with Epstein-Barr virus and a propensity for the development of lymphoma in adulthood.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
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.
Tissue Specificity
Expressed most strongly in peripheral blood leukocytes, the spleen, and the thymus.
Cellular localization
- Nucleus
- Cytoplasm
- Isoform 1
- Nucleus
- Isoform 3
- Nucleus
- Isoform 11
- Nucleus
- Isoform 14
- Nucleus
- Cytoplasm
- Isoform 12
- Cytoplasm
Alternative names
ZNFN1A3, IKZF3, Zinc finger protein Aiolos, Ikaros family zinc finger protein 3