PMID- 10572087
OWN - NLM
STAT- MEDLINE
DCOM- 19991230
LR  - 20171116
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 94
IP  - 11
DP  - 1999 Dec 1
TI  - A novel BTB/POZ transcriptional repressor protein interacts with the Fanconi
      anemia group C protein and PLZF.
PG  - 3737-47
AB  - Fanconi anemia (FA) is an autosomal recessive cancer susceptibility syndrome. The
      phenotype includes developmental defects, bone marrow failure, and cell cycle
      abnormalities. At least eight complementation groups (A-H) exist, and although
      three of the corresponding complementation group genes have been cloned, they
      lack recognizable motifs, and their functions are unknown. We have isolated a
      binding partner for the Fanconi anemia group C protein (FANCC) by yeast
      two-hybrid screening. We show that the novel gene, FAZF, encodes a 486 amino acid
      protein containing a conserved amino terminal BTB/POZ protein interaction domain 
      and three C-terminal Kruppel-like zinc fingers. FAZF is homologous to the
      promyelocytic leukemia zinc finger (PLZF) protein, which has been shown to act as
      a transcriptional repressor by recruitment of nuclear corepressors (N-CoR, Sin3, 
      and HDAC1 complex). Consistent with a role in FA, BTB/POZ-containing proteins
      have been implicated in oncogenesis, limb morphogenesis, hematopoiesis, and
      proliferation. We show that FAZF is a transcriptional repressor that is able to
      bind to the same DNA target sequences as PLZF. Our data suggest that the
      FAZF/FANCC interaction maps to a region of FANCC deleted in FA patients with a
      severe disease phenotype. We also show that FAZF and wild-type FANCC can
      colocalize in nuclear foci, whereas a patient-derived mutant FANCC that is
      compromised for nuclear localization cannot. These results suggest that the
      function of FANCC may be linked to a transcriptional repression pathway involved 
      in chromatin remodeling.
FAU - Hoatlin, M E
AU  - Hoatlin ME
AD  - Division of Hematology and Medical Oncology, Oregon Health Sciences University,
      Portland, OR 97201, USA. hoatlinm@OHSU.edu
FAU - Zhi, Y
AU  - Zhi Y
FAU - Ball, H
AU  - Ball H
FAU - Silvey, K
AU  - Silvey K
FAU - Melnick, A
AU  - Melnick A
FAU - Stone, S
AU  - Stone S
FAU - Arai, S
AU  - Arai S
FAU - Hawe, N
AU  - Hawe N
FAU - Owen, G
AU  - Owen G
FAU - Zelent, A
AU  - Zelent A
FAU - Licht, J D
AU  - Licht JD
LA  - eng
GR  - CA59938/CA/NCI NIH HHS/United States
GR  - HL56045/HL/NHLBI NIH HHS/United States
GR  - K08 CA73762/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
RN  - 0 (ABTB1 protein, human)
RN  - 0 (Cell Cycle Proteins)
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (FANCC protein, human)
RN  - 0 (Fanconi Anemia Complementation Group C Protein)
RN  - 0 (Fanconi Anemia Complementation Group Proteins)
RN  - 0 (Kruppel-Like Transcription Factors)
RN  - 0 (Nuclear Proteins)
RN  - 0 (Promyelocytic Leukemia Zinc Finger Protein)
RN  - 0 (Proteins)
RN  - 0 (Repressor Proteins)
RN  - 0 (Transcription Factors)
RN  - 147855-37-6 (ZBTB16 protein, human)
SB  - AIM
SB  - IM
MH  - Amino Acid Sequence
MH  - Base Sequence
MH  - *Cell Cycle Proteins
MH  - DNA-Binding Proteins/*genetics/metabolism
MH  - Fanconi Anemia/*genetics/metabolism
MH  - Fanconi Anemia Complementation Group C Protein
MH  - Fanconi Anemia Complementation Group Proteins
MH  - Gene Expression Regulation
MH  - Humans
MH  - Kruppel-Like Transcription Factors
MH  - Molecular Sequence Data
MH  - *Nuclear Proteins
MH  - Promyelocytic Leukemia Zinc Finger Protein
MH  - Proteins/*genetics/metabolism
MH  - Repressor Proteins/*genetics/metabolism
MH  - Transcription Factors/*genetics/metabolism
EDAT- 1999/11/26 00:00
MHDA- 1999/11/26 00:01
CRDT- 1999/11/26 00:00
PHST- 1999/11/26 00:00 [pubmed]
PHST- 1999/11/26 00:01 [medline]
PHST- 1999/11/26 00:00 [entrez]
PST - ppublish
SO  - Blood. 1999 Dec 1;94(11):3737-47.