| Description | 540 and 673 amino acids, 62 and 74 kDa (PLZFa and b respectively); Kr¸ppel-like zinc finger protein; N-term BTB/POZ domain (protein-protein interaction motif) and 9 zinc finger domains in C-term |
| Expression | highly specific for hematopoietic cells; during mouse development, PLZF is expressed in embryonic tissus, giving rise to hematopoietic progenitors; in man, PLZF is expressed in CD34+ progenitor cells and in primitive multipotent hematopoietic cell lines; PLZF levels decrease progressively with cell maturation and also with maturation induced by ATRA treatment. |
| Localisation | nuclear; immunodetection shows a nuclear speckeled distribution of PLZF, resulting from a localization on discrete nuclear bodies whose formation is dependent on the integrity of the BTB/POZ domain. |
| Function | putative transcription factor associated with myeloid differentiation; sequence specific DNA binding activity; in co-transfection experiments, PLZF has been shown to represses the transcription. |
| Homology | with the zinc finger MZF-1 |
| Entity | t(11;17)(q23;q21) in M3 ANLL --> PLZF - RARA |
| Disease | acute promyelocytic leukaemia with a 'variant' translocation |
| Prognosis | worse prognosis than M3 ANLL with t(15;17), because the patients fail to respond to the maturation effect of ATRA |
| Cytogenetics | t(11;17), like t(15;17)(q22;q21), and like another t(11;17)(q13;q21), with a discrete breakpoint in 11q13 are, closely related to t(15;17)(q22;q21) |
| Hybrid/Mutated Gene | both fusion transcripts are expressed |
| Abnormal Protein | the two fusion proteins are produced; the PLZF/RARa protein contains the N-terminal BTB/POZ domain and two out of the nine zinc finger domains from PLZF; tThe POZ domain is largely responsible for the activity of PLZF/RARa fusion protein. |
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| Rearrangements of the retinoic acid receptor alpha and promyelocytic leukemia zinc finger genes resulting from t(11;17)(q23;q21) in a patient with acute promyelocytic leukemia. |
| Chen SJ, Zelent A, Tong JH, Yu HQ, Wang ZY, Derrˆ© J, Berger R, Waxman S, Chen Z |
| The Journal of clinical investigation. 1993 ; 91 (5) : 2260-2267. |
| PMID 8387545 |
| |
| Reduced and altered DNA-binding and transcriptional properties of the PLZF-retinoic acid receptor-alpha chimera generated in t(11;17)-associated acute promyelocytic leukemia. |
| Licht JD, Shaknovich R, English MA, Melnick A, Li JY, Reddy JC, Dong S, Chen SJ, Zelent A, Waxman S |
| Oncogene. 1996 ; 12 (2) : 323-336. |
| PMID 8570209 |
| |
| PML, PLZF and NPM genes in the molecular pathogenesis of acute promyelocytic leukemia. |
| Pandolfi PP |
| Haematologica. 1996 ; 81 (5) : 472-482. |
| PMID 8952164 |
| |
| Leukemia-associated retinoic acid receptor alpha fusion partners, PML and PLZF, heterodimerize and colocalize to nuclear bodies. |
| Koken MH, Reid A, Quignon F, Chelbi-Alix MK, Davies JM, Kabarowski JH, Zhu J, Dong S, Chen S, Chen Z, Tan CC, Licht J, Waxman S, de Thˆ© H, Zelent A |
| Proceedings of the National Academy of Sciences of the United States of America. 1997 ; 94 (19) : 10255-10260. |
| PMID 9294197 |
| |
