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protein CBFA2T3 (myeloid translocation gene on chromosome 16 protein, hMTG16, MTG8-related protein 2, zinc finger MYND domain-containing protein 4, CBFA2T3, MTG16, MTGR2, ZMYND4)
Function:
- transcriptional repressor
- regulates proliferation & differentiation of erythroid progenitors by repressing the expression of TAL1 target genes
- role in granulocyte differentiation
- isoform 2 functions as an A-kinase-anchoring protein [2]
- component of a TAL-1 complex
- heterodimer with RUNX1T1 & CBFA2T2
- interacts with ERBB4, HDAC1, HDAC2, HDAC3, HDAC6,* HDAC8, * interaction with HDAC6 uncertain
- isoform 2 interacts with PRKAR2A, PDE7A & probably PDE4A
Structure:
- belongs to the CBFA2T family
- contains 1 MYND-type zinc finger
- contains 1 TAFH (NHR1) domain
Compartment:
- isoform 1: nucleus, nucleolus
- the RUNX1-CBFA2T3 fusion protein localizes to the nucleoplasm
- isoform 2: nucleus, nucleoplasm, Golgi membrane
Alternative splicing: named isoforms=5
Expression:
- widely expressed
- higher expression in heart, pancreas, skeletal muscle, spleen, thymus & peripheral blood leukocytes
- expressed in hematopoietic cells (at protein level)
- down-regulated by all-trans retinoic acid
Pathology:
- chromosomal translocation t(16;21)(q24;q22) involving CBFA2T3 with RUNX1 is found in therapy-related myeloid malignancies
- translocation forms a RUNX1-CBFA2T3 fusion protein
General
zinc finger protein
Properties
SIZE: entity length = 653 aa
MW = 71 kD
MOTIF: Mediates localization to the nucleus {1-435}
MOTIF: PDE7A interaction {1-430}
nucleolar targeting {1-127}
proline-rich region
SITE: 42-99
MOTIF: proline residue (SEVERAL)
breakpoint {51-52}
breakpoint {127-128}
TAFH {171-266}
mediates interaction with PRKAR2A {485-506}
coiled coil {488-543}
Zinc finger
NAME: Zinc finger
SITE: 556-592
EFFECTOR-BOUND: Zn+2
Database Correlations
OMIM 603870
UniProt O75081
PFAM correlations
References
- UniProt :accession O75081
- Schillace RV, Andrews SF, Liberty GA, Davey MP, Carr DW.
Identification and characterization of myeloid translocation
gene 16b as a novel a kinase anchoring protein in
T lymphocytes.
J Immunol. 2002 Feb 15;168(4):1590-9.
PMID: 11823486
- Hoogeveen AT et al
The transcriptional corepressor MTG16a contains a novel nucleolar
targeting sequence deranged in t (16; 21)-positive myeloid
malignancies.
Oncogene. 2002 Sep 26;21(43):6703-12.
PMID: 12242670
- Atlas of Genetics & Cytogenetics in Oncology & Haematology
http://atlasgeneticsoncology.org/Genes/CBFA2T3ID428ch16q24.html
Component-of
TAL1 complex