Database: OMIM
Entry: 176797
LinkDB: 176797
MIM Entry: 176797
  Chen et al. (1993) identified the PLZF gene on chromosome 11 as the
  fusion partner of the retinoic acid receptor-alpha gene (RARA; 180240)
  on chromosome 17 in a Chinese patient with acute promyelocytic leukemia
  (APL; 612376) and a translocation t(11;17)(q23;21). Chen et al. (1993)
  described the PLZF gene.
  Reid et al. (1995) showed that murine PLZF is expressed at highest
  levels in undifferentiated, multipotential hematopoietic progenitor
  cells and its expression declines as cells become more mature and
  committed to various hematopoietic lineages. In the human there is a
  lack of PLZF protein expression in mature peripheral blood mononuclear
  cells and high PLZF levels in the nuclei of CD34+ human bone marrow
  progenitor cells. Unlike many transcription factors, PLZF protein in
  these cells shows a distinct punctate distribution, suggesting its
  compartmentalization in the nucleus.
  Zhang et al. (1999) identified at least 4 alternative splicings (AS-I,
  -II, -III, and -IV) within exon 1 of the PLZF gene. AS-I was detected in
  most tissues tested, whereas AS-II, -III, and -IV were present in the
  stomach, testis, and heart, respectively. Although splicing donor and
  acceptor signals at exon-intron boundaries for AS-I and exons 1-6 were
  classic (gt-ag), AS-II, -III, and -IV had atypical splicing sites. These
  alternative splicings, nevertheless, maintained the open reading frame
  and may encode isoforms with absence of important functional domains. In
  mRNA species without AS-I, there is a relatively long 5-prime UTR of 6.0
  kb. Zhang et al. (1999) determined that PLZF is a well-conserved gene
  from C. elegans to human. PLZF paralogous sequences are found in the
  human genome. The presence of 2 MLL/PLZF-like alignments on human
  chromosomes 11q23 and 19 suggests a syntenic replication during
  Kang et al. (2003) found that endogenous PLZF in a human promyelocytic
  cell line was modified by conjugation with SUMO1 (601912) and that PLZF
  colocalized with SUMO1 in the nucleus of transfected human embryonic
  kidney cells. Site-directed mutagenesis identified lys242 in
  transcriptional repression domain-2 as the site of PLZF sumoylation.
  Reporter gene assays suggested that SUMO1 modification of lys242 was
  required for transcriptional repression by PLZF, and electrophoretic
  mobility shift assays showed sumoylation increased the DNA-binding
  activity of PLZF. PLZF-mediated regulation of the cell cycle and
  transcriptional repression of the cyclin A2 gene (CCNA2; 123835) were
  also dependent on sumoylation of PLZF on lys242.
  Ikeda et al. (2005) found that PLZF was 1 of 24 genes upregulated during
  osteoblastic differentiation of cultured OPLL (602475) ligament cells.
  PLZF was highly expressed during osteoblastic differentiation in all
  ligament and mesenchymal stem cells examined. Silencing of the PLZF gene
  by small interfering RNA in human and mouse mesenchymal stem cells
  reduced expression of osteoblast-specific genes, such as alkaline
  phosphatase (ALPL; 171760), collagen 1A1 (COL1A1; 120150), Cbfa1 (RUNX2;
  600211), and osteocalcin (BGLAP; 112260). PLZF expression was unaffected
  by the addition of BMP2 (112261), and BMP2 expression was not affected
  by PLZF expression. In a mouse mesenchymal cell line, overexpression of
  PLZF increased expression of Cbfa1 and Col1a1; on the other hand, CBFA1
  overexpression did not affect expression of Plzf. Ikeda et al. (2005)
  concluded that PLZF plays a role in early osteoblastic differentiation
  and is an upstream regulator of CBFA1.
  Using yeast 2-hybrid analysis and protein pull-down assays, Rho et al.
  (2006) showed that PLZF interacted with the CCS3 isoform of EEF1A1
  (130590). Mutation analysis revealed that repressor domain-2 and the
  zinc finger domain of PLZF were required for the interaction. CCS3 was
  required for the transcriptional effects of PLZF in reporter gene
  Tissing et al. (2007) found that 8 hours of prednisolone treatment
  altered expression of 51 genes in leukemic cells from children with
  precursor-B- or T-acute lymphoblastic leukemia compared with nonexposed
  cells. The 3 most highly upregulated genes were FKBP5 (602623), ZBTB16,
  and TXNIP (606599), which were upregulated 35.4-, 8.8-, and 3.7-fold,
  Using microarray analysis, Good and Tangye (2007) showed that naive
  splenic B cells expressed higher levels of transcription factors KLF4
  (602253), KLF9 (602902), and PZLF compared with memory B cells.
  Activation of naive B cells through CD40 (109535) and B-cell receptor
  downregulated expression of these cellular quiescence-associated
  transcription factors. Overexpression of KLF4, KLF9, and PZLF in memory
  B cells delayed their entry into cell division and proliferation. Good
  and Tangye (2007) concluded that memory B cells undergo a rewiring
  process that results in a significantly reduced activation threshold
  compared with naive B cells, allowing them to enter division more
  quickly, to differentiate into Ig-secreting plasma cells, and to more
  rapidly produce antibodies.
  - PLZF/RARA Fusion Protein
  Chen et al. (1994) cloned cDNAs encoding PLZF-RARA chimeric proteins and
  studied their transactivating activities. A 'dominant-negative' effect
  was observed when PLZF-RARA fusion proteins were cotransfected with
  vectors expressing RARA and retinoid X receptor alpha (RXRA; 180245).
  These abnormal transactivation properties observed in retinoic
  acid-sensitive myeloid cells strongly implicated the fusion proteins in
  the molecular pathogenesis of acute promyelocytic leukemia (APL;
  Lin et al. (1998) reported that the association of PLZF-RAR-alpha and
  PML-RAR-alpha (see 102578) with the histone deacetylase complex (see
  605164) helps to determine both the development of APL and the ability
  of patients to respond to retinoids. Consistent with these observations,
  inhibitors of histone deacetylase dramatically potentiate
  retinoid-induced differentiation of retinoic acid-sensitive, and restore
  retinoid responses of retinoic acid-resistant, APL cell lines. Lin et
  al. (1998) concluded that oncogenic retinoic acid receptors mediate
  leukemogenesis through aberrant chromatin acetylation, and that
  pharmacologic manipulation of nuclear receptor cofactors may be a useful
  approach in the treatment of human disease.
  Grignani et al. (1998) demonstrated that both PML-RAR-alpha and
  PLZF-RAR-alpha fusion proteins recruit the nuclear corepressor (NCOR;
  see 600849)-histone deacetylase complex through the RAR-alpha CoR box.
  PLZF-RAR-alpha contains a second, retinoic acid-resistant binding site
  in the PLZF amino-terminal region. High doses of retinoic acid release
  histone deacetylase activity from PML-RAR-alpha, but not from
  PLZF-RAR-alpha. Mutation of the NCOR binding site abolishes the ability
  of PML-RAR-alpha to block differentiation, whereas inhibition of histone
  deacetylase activity switches the transcriptional and biologic effects
  of PLZF-RAR-alpha from being an inhibitor to an activator of the
  retinoic acid signaling pathway. Therefore, Grignani et al. (1998)
  concluded that recruitment of histone deacetylase is crucial to the
  transforming potential of APL fusion proteins, and the different effects
  of retinoic acid on the stability of the PML-RAR-alpha and
  PLZF-RAR-alpha corepressor complexes determines the differential
  response of APLs to retinoic acid.
  Guidez et al. (2007) identified CRABP1 (180230) as a target of both PLZF
  and the RARA/PLZF fusion protein. PLZF repressed CRABP1 through
  propagation of chromatin condensation from a remote intronic binding
  element, culminating in silencing of the CRABP1 promoter. Although the
  canonical PLZF/RARA oncoprotein had no effect on PLZF-mediated
  repression, the reciprocal translocation product, RARA/PLZF, bound to
  this remote binding site, recruited p300 (EP300; 602700), and induced
  promoter hypomethylation and CRABP1 upregulation. Similarly, retinoic
  acid-resistant murine blasts that expressed both fusion proteins
  expressed much higher levels of Crabp1 than retinoic acid-sensitive
  cells expressing Plzf/Rara alone. RARA/PLZF conferred retinoic acid
  resistance to a retinoid-sensitive acute myeloid leukemia cell line in a
  CRABP1-dependent fashion. Guidez et al. (2007) concluded that
  upregulation of CRABP1 by RARA/PLZF contributes to retinoid resistance
  in leukemia.
  Ahmad et al. (1998) reported the crystal structure of the BTB domain of
  PLZF. The BTB domain (also known as the POZ domain) is an evolutionarily
  conserved protein-protein interaction motif found at the N terminus of 5
  to 10% of C2H2-type zinc finger transcription factors. The BTB domain
  has transcriptional repression activity and interacts with components of
  the histone deacetylase complex. The latter association provides a
  mechanism of linking the transcription factor with enzymatic activities
  that regulate chromatin conformation.
  Zhang et al. (1999) sequenced a 201-kb genomic DNA region containing the
  entire PLZF gene. Repeated elements accounted for 19.83%, and no obvious
  coding information other than PLZF was present in this region. PLZF was
  found to contain 6 exons and 5 introns, and the exon organization
  corresponded well with protein domains. Zhang et al. (1999) identified
  at least 4 alternative splicings (AS-I, -II, -III, and -IV) within exon
  Van Schothorst et al. (1999) determined that the ZNF145 gene contains 7
  exons and spans at least 120 kb. The untranslated exon 1 is located
  within a CpG island, and several SP1 (189906)- and GATA1
  (305371)-binding sites are upstream of exon 1.
  By FISH, Chen et al. (1993) localized the PLZF gene to chromosome
  Almost all patients with acute promyelocytic leukemia (APL; 612376) have
  a chromosomal translocation t(15;17)(q22;q21). Molecular studies reveal
  that the translocation results in a chimeric gene through fusion between
  the promyelocytic leukemia gene (PML; 102578) on chromosome 15 and the
  retinoic acid receptor-alpha gene (RARA; 180240) on chromosome 17. Chen
  et al. (1993) reported studies of a Chinese patient with APL and a
  variant translocation t(11;17)(q23;21) in which the PLZF gene on
  chromosome 11q23.1 was fused to the RARA gene on chromosome 17. Similar
  to t(15;17) APL, all-trans retinoic acid treatment produced an early
  leukocytosis which was followed by a myeloid maturation, but the patient
  died too early to achieve remission.
  Zhang et al. (1999) characterized the chromosomal breakpoints and
  joining sites in the index acute promyelocytic leukemia case with
  t(11;17), reported by Chen et al. (1993). The results suggested the
  involvement of a DNA damage-repair mechanism.
  In a 12.75-year-old boy with skeletal defects, genital hypoplasia, and
  mental retardation (612447), originally reported by Wieczorek et al.
  (2002), Fischer et al. (2008) performed array-based CGH and identified
  an approximately 8-Mb de novo deletion on the paternal chromosome 11, a
  region containing about 72 genes. Sequence analysis of the candidate
  gene ZBTB16 on the maternal allele revealed a missense mutation (M617V;
  176797.0001); reporter gene assays showed that the mutation impairs
  ZBTB16 function. No ZBTB16 mutation was found in 41 patients who had
  clinical overlap with this patient, including patients with severe
  hypoplasia of forearms.
  Cheng et al. (1999) generated transgenic mice with PLZF-RARA and NPM
  (164040)-RARA. PLZF-RARA transgenic animals developed chronic myeloid
  leukemia-like phenotypes at an early stage in life (within 3 months in 5
  of 6 mice), whereas 3 NPM-RARA transgenic mice showed a spectrum of
  phenotypes from typical APL to chronic myeloid leukemia relatively late
  in life (from 12 to 15 months). In contrast to bone marrow cells from
  PLZF-RARA transgenic mice, those from NPM-RARA transgenic mice could be
  induced to differentiate by all-trans-retinoic acid (ATRA). Cheng et al.
  (1999) found that in interacting with nuclear coreceptors the 2 fusion
  proteins had different ligand sensitivities, which may be the underlying
  molecular mechanism for differential responses to ATRA. These data
  clearly established the leukemogenic role of PLZF-RARA and NPM-RARA and
  the importance of fusion receptor/corepressor interactions in the
  pathogenesis as well as in determining different clinical phenotypes of
  He et al. (2000) generated transgenic mice expressing RARA-PLZF and
  PLZF-RARA in their promyelocytes. RARA-PLZF transgenic mice did not
  develop leukemia. However, PLZF-RARA/RARA-PLZF double transgenic mice
  developed leukemia with classic APL features. The authors demonstrated
  that RARA-PLZF can interfere with PLZF transcriptional repression, and
  that this is critical for APL pathogenesis, since leukemias in
  PLZF-deficient/PLZF-RARA mutants and in PLZF-RARA/RARA-PLZF transgenic
  mice were indistinguishable. Thus, both products of a cancer-associated
  translocation are crucial in determining the distinctive features of the
  Barna et al. (2000) generated Zfp145 -/- mice and showed that Plzf is
  essential for patterning of the limb and axial skeleton. Inactivation of
  the gene resulted in patterning defects affecting all skeletal
  structures of the limb, including homeotic transformations of anterior
  skeletal elements into posterior structures. They demonstrated that Plzf
  acts as a growth-inhibitory and proapoptotic factor in the limb bud. The
  expression of members of the Abdominal B (Abdb) Hox gene complex (see
  142956), as well as genes encoding bone morphogenetic proteins (e.g.,
  112267), was altered in the developing limb of the Zfp145 -/- mice. The
  mice also exhibited anterior-directed homeotic transformation throughout
  the axial skeleton with associated alterations in Hox gene expression.
  Plzf is, therefore, a mediator of anterior-to-posterior patterning in
  both the axial and appendicular skeleton and acts as a regulator of Hox
  gene expression.
  Barna et al. (2002) determined that the defects in Plzf -/- mice were
  due to spatial, but not temporal, deregulation of the Abdb Hoxd complex.
  They identified several Plzf-binding sites in Hoxd11 (142986) and showed
  that Plzf bound Hoxd11 genomic DNA fragments as a dimer or possibly a
  trimer, mostly when DNA loops were formed. Barna et al. (2002) also
  found evidence of long-range interactions between distant Plzf-binding
  sites within the Hoxd regulatory elements. Plzf mediated transcriptional
  repression of a Hoxd reporter construct, and in the absence of Plzf,
  there were increased acetylated histones on Hoxd regulatory regions.
  Plzf showed dose-dependent transcriptional repression of a Hoxd reporter
  in mouse anterior limb micromass cultures, but there was no repression
  in posterior limb micromass cultures. Plzf also directly tethered the
  polycomb protein Bmi1 (164831) on DNA, which antagonized posteriorizing
  signals in the limb. Barna et al. (2002) concluded that recruitment of
  histone deacetylases and polycomb proteins by PLZF favors transition
  from euchromatin to heterochromatin.
  Adult germline stem cells are capable of self-renewal, tissue
  regeneration, and production of large numbers of differentiated progeny.
  The mouse mutant 'luxoid' (lu) arose spontaneously and was mapped to
  mouse chromosome 9 (Green, 1955), and was initially characterized by its
  semidominant abnormalities and recessive skeletal and male infertility
  phenotypes (Forsthoefel, 1958). Buaas et al. (2004) showed that the
  mouse mutant luxoid affects adult germline stem cell self-renewal. Young
  homozygous luxoid mutant mice produce limited numbers of normal
  spermatozoa and then progressively lose their germline after birth.
  Transplantation studies showed that germ cells of mutant mice did not
  colonize recipient testes, suggesting that the defect is intrinsic to
  the stem cells. Buaas et al. (2004) determined that the luxoid mutant
  contains a nonsense mutation in the Plzf gene, a transcriptional
  repressor that regulates the epigenetic state of undifferentiated cells.
  They showed, furthermore, that Plzf is coexpressed with Oct4 (164177) in
  undifferentiated spermatogonia. This was said to be the first gene found
  to be required in germ cells for stem cell self-renewal in mammals.
  Costoya et al. (2004) likewise showed that Plzf has a crucial role in
  spermatogenesis. Expression of the gene was restricted to gonocytes and
  undifferentiated spermatogonia and was absent in the tubules of W/W(v)
  mutants that lack these cells. Mice lacking Plzf underwent a progressive
  loss of spermatogonia with age, associated with increases in apoptosis
  and subsequent loss of tubule structure but without overt
  differentiation defects or loss of the supporting Sertoli cells.
  Spermatogonia transplantation experiments revealed a depletion of
  spermatogonia stem cells in the adult. These and other results
  identified Plzf as a spermatogonia-specific transcription factor in the
  testis that is required to regulate self-renewal and maintenance of the
  stem cell pool.
  Barna et al. (2005) identified a genetic interaction between Gli3
  (165240) and Plzf that is required specifically at very early stages of
  limb development for all proximal cartilage condensations in the
  hindlimb (femur, tibia, fibula). Notably, distal condensations
  comprising the foot were relatively unperturbed in Gli3/Plzf double
  knockout mouse embryos. Barna et al. (2005) demonstrated that the
  cooperative activity of Gli3 and Plzf establishes the correct temporal
  and spatial distribution of chondrocyte progenitors in the proximal limb
  bud independently of proximal-distal (P-D) patterning markers and
  overall limb bud size. Moreover, the limb defects in the double knockout
  embryos correlated with the transient death of a specific subset of
  proximal mesenchymal cells that express bone morphogenetic protein
  receptor type 1B (Bmpr1b; 603248) at the onset of limb development.
  Barna et al. (2005) concluded that development of proximal and distal
  skeletal elements is distinctly regulated during early limb bud
  formation. The initial division of the vertebrate limb into 2 distinct
  molecular domains is consistent with fossil evidence indicating that the
  upper and lower extremities of the limb have different evolutionary
Allelic Variants:
  In a 12.75-year-old boy with skeletal defects, genital hypoplasia, and
  mental retardation (612447), originally reported by Wieczorek et al.
  (2002), Fischer et al. (2008) performed array-based CGH and identified
  an approximately 8-Mb de novo deletion on the paternal chromosome 11.
  Sequence analysis of the candidate gene ZBTB16 on the maternal allele
  revealed a 1849A-G transition in exon 6, resulting in a met617-to-val
  (M617V) substitution at a highly conserved residue within the eighth
  zinc finger motif of the PLZF protein, predicted to destabilize the
  alpha helix of the zinc finger that forms the contact with the DNA
  duplex. Reporter gene assays showed that the mutant PLZF decreased
  luciferase activity by only 10%, compared to an approximately 35%
  decrease with wildtype PLZF, suggesting that this represents a
  hypomorphic allele. The mutation was not found in 200 normal control
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  Marla J. F. O'Neill - updated: 12/1/2008
  Paul J. Converse - updated: 10/27/2008
  Patricia A. Hartz - updated: 5/1/2008
  Patricia A. Hartz - updated: 2/28/2008
  Patricia A. Hartz - updated: 11/29/2007
  Patricia A. Hartz - updated: 9/2/2005
  Ada Hamosh - updated: 8/18/2005
  Victor A. McKusick - updated: 6/14/2004
  Ada Hamosh - updated: 5/1/2001
  Ada Hamosh - updated: 4/30/2001
  Stylianos E. Antonarakis - updated: 12/14/2000
  Victor A. McKusick - updated: 5/27/2000
  Victor A. McKusick - updated: 10/21/1999
  Victor A. McKusick - updated: 7/13/1999
  Victor A. McKusick - updated: 11/3/1998
Creation Date: 
  Victor A. McKusick: 6/4/1993
Edit Dates: 
  carol: 12/02/2008
  carol: 12/1/2008
  terry: 12/1/2008
  mgross: 10/28/2008
  mgross: 10/27/2008
  mgross: 5/1/2008
  mgross: 2/28/2008
  mgross: 11/30/2007
  terry: 11/29/2007
  carol: 11/22/2006
  mgross: 9/6/2005
  terry: 9/2/2005
  alopez: 8/23/2005
  terry: 8/18/2005
  tkritzer: 6/29/2004
  terry: 6/14/2004
  carol: 6/20/2001
  alopez: 5/1/2001
  alopez: 4/30/2001
  mgross: 12/14/2000
  alopez: 5/27/2000
  carol: 10/22/1999
  terry: 10/21/1999
  psherman: 7/27/1999
  mgross: 7/19/1999
  terry: 7/13/1999
  carol: 11/13/1998
  carol: 11/9/1998
  terry: 11/3/1998
  dkim: 9/25/1998
  dkim: 9/11/1998
  mark: 3/11/1996
  terry: 3/4/1996
  mimadm: 2/25/1995
  carol: 4/14/1994
  carol: 11/9/1993
  carol: 7/9/1993
  carol: 6/4/1993
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