scholarly journals Fanconi Anemia Group C Protein

2020 ◽  
Author(s):  
Keyword(s):  
Fanconi Anemia ◽  
C Protein ◽  
Anemia Group

A Novel BTB/POZ Transcriptional Repressor Protein Interacts With the Fanconi Anemia Group C Protein and PLZF

Blood ◽  
1999 ◽  
Vol 94 (11) ◽  
pp. 3737-3747 ◽  
Author(s):  
Maureen E. Hoatlin ◽  
Yu Zhi ◽  
Helen Ball ◽  
Kirsten Silvey ◽  
Ari Melnick ◽  
...  
Keyword(s):  
Fanconi Anemia ◽  
Transcriptional Repression ◽  
Cancer Susceptibility ◽  
Bone Marrow Failure ◽  
Severe Disease ◽  
Transcriptional Repressor ◽  
Interaction Domain ◽  
C Protein ◽  
Amino Terminal ◽  
Anemia Group

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 Krüppel-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.

scholarly journals The Fanconi Anemia Group C Protein Interacts with Uncoordinated 5A and Delays Apoptosis

PLoS ONE ◽  
2014 ◽  
Vol 9 (3) ◽  
pp. e92811 ◽  
Author(s):  
FengFei Huang ◽  
Manel Ben Aissa ◽  
Audrey Magron ◽  
Caroline C. Huard ◽  
Chantal Godin ◽  
...  
Keyword(s):  
Fanconi Anemia ◽  
C Protein ◽  
Anemia Group

scholarly journals Regulation of the Fanconi Anemia Group C Protein through Proteolytic Modification

2003 ◽  
Vol 279 (6) ◽  
pp. 4713-4720 ◽  
Author(s):  
Isabelle Brodeur ◽  
Isabelle Goulet ◽  
Cédric S. Tremblay ◽  
Chantal Charbonneau ◽  
Marie-Chantal Delisle ◽  
...  
Keyword(s):  
Fanconi Anemia ◽  
C Protein ◽  
Anemia Group

Fanconi anemia group C protein prevents apoptosis in hematopoietic cells through redox regulation of GSTP1

10.1038/89937 ◽  
2001 ◽  
Vol 7 (7) ◽  
pp. 814-820 ◽  
Author(s):  
Robert C. Cumming ◽  
Jeff Lightfoot ◽  
Kristin Beard ◽  
Hagop Youssoufian ◽  
Peter J. O'Brien ◽  
...  
Keyword(s):  
Fanconi Anemia ◽  
Redox Regulation ◽  
Hematopoietic Cells ◽  
C Protein ◽  
Anemia Group

A Novel BTB/POZ Transcriptional Repressor Protein Interacts With the Fanconi Anemia Group C Protein and PLZF

Blood ◽  
1999 ◽  
Vol 94 (11) ◽  
pp. 3737-3747 ◽  
Author(s):  
Maureen E. Hoatlin ◽  
Yu Zhi ◽  
Helen Ball ◽  
Kirsten Silvey ◽  
Ari Melnick ◽  
...  
Keyword(s):  
Fanconi Anemia ◽  
Transcriptional Repression ◽  
Cancer Susceptibility ◽  
Bone Marrow Failure ◽  
Severe Disease ◽  
Transcriptional Repressor ◽  
Interaction Domain ◽  
C Protein ◽  
Amino Terminal ◽  
Anemia Group

Abstract 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 Krüppel-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.

scholarly journals Functional Correction of Fanconi Anemia Group C Hematopoietic Cells by the Use of a Novel Lentiviral Vector

2001 ◽  
Vol 3 (4) ◽  
pp. 485-490 ◽  
Author(s):  
Kaoru Yamada ◽  
John C. Olsen ◽  
Manij Patel ◽  
Kathleen W. Rao ◽  
Christopher E. Walsh
Keyword(s):  
Fanconi Anemia ◽  
Lentiviral Vector ◽  
Hematopoietic Cells ◽  
Functional Correction ◽  
Anemia Group

scholarly journals Engraftment of Hematopoietic Progenitor Cells Transduced with the Fanconi Anemia Group C Gene (FANCC)

1999 ◽  
Vol 10 (14) ◽  
pp. 2337-2346 ◽  
Author(s):  
Johnson M. Liu ◽  
Sonnie Kim ◽  
Elizabeth J. Read ◽  
Makoto Futaki ◽  
Inderjeet Dokal ◽  
...  
Keyword(s):  
Progenitor Cells ◽  
Fanconi Anemia ◽  
Hematopoietic Progenitor Cells ◽  
Hematopoietic Progenitor ◽  
Anemia Group

Fanconi anemia group A and C double-mutant mice

2002 ◽  
Vol 30 (7) ◽  
pp. 679-688 ◽  
Author(s):  
Meenakshi Noll ◽  
Kevin P Battaile ◽  
Raynard Bateman ◽  
Timothy P Lax ◽  
Keany Rathbun ◽  
...  
Keyword(s):  
Fanconi Anemia ◽  
Double Mutant ◽  
Mutant Mice ◽  
Group A ◽  
Anemia Group
Sign in / Sign up

Export Citation Format

Share Document