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The Fanconi anemia pathway has a dual function in Dickkopf-1 transcriptional repression.

Fanconi anemia (FA) is an inherited bone marrow failure syndrome associated with a progressive decline in hematopoietic stem cells, developmental defects, and predisposition to cancer. These various phenotypic features imply a role of FA proteins in molecular events regulating cellular homeostasis.... Full description

Journal Title: Proceedings of the National Academy of Sciences of the United States of America February 11, 2014, Vol.111(6), pp.2152-2157
Main Author: Huard, Caroline C
Other Authors: Tremblay, Cédric S , Magron, Audrey , Lévesque, Georges , Carreau, Madeleine
Format: Electronic Article Electronic Article
Language: English
Subjects:
ID: E-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1314226111
Link: http://search.proquest.com/docview/1499139595/?pq-origsite=primo
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title: The Fanconi anemia pathway has a dual function in Dickkopf-1 transcriptional repression.
format: Article
creator:
  • Huard, Caroline C
  • Tremblay, Cédric S
  • Magron, Audrey
  • Lévesque, Georges
  • Carreau, Madeleine
subjects:
  • Enzyme Activation–Genetics
  • Fanconi Anemia–Metabolism
  • Fanconi Anemia Complementation Group C Protein–Metabolism
  • Gene Expression Regulation–Metabolism
  • Glycogen Synthase Kinase 3–Genetics
  • Glycogen Synthase Kinase 3 Beta–Genetics
  • Hek293 Cells–Genetics
  • Humans–Genetics
  • Intercellular Signaling Peptides and Proteins–Genetics
  • Transcription, Genetic–Genetics
  • Dkk1 Protein, Human
  • Fanconi Anemia Complementation Group C Protein
  • Intercellular Signaling Peptides and Proteins
  • Gsk3b Protein, Human
  • Glycogen Synthase Kinase 3 Beta
  • Glycogen Synthase Kinase 3
ispartof: Proceedings of the National Academy of Sciences of the United States of America, February 11, 2014, Vol.111(6), pp.2152-2157
description: Fanconi anemia (FA) is an inherited bone marrow failure syndrome associated with a progressive decline in hematopoietic stem cells, developmental defects, and predisposition to cancer. These various phenotypic features imply a role of FA proteins in molecular events regulating cellular homeostasis. Interestingly, we previously found that the Fanconi C protein (FANCC) interacts with the C-terminal-binding protein-1 (CtBP1) involved in transcriptional regulation. Here we report that FANCC with CtBP1 forms a complex with [beta]-catenin, and that [beta]-catenin activation through glycogen synthase kinase 3[beta] inhibition leads to FANCC nuclear accumulation and FA pathway activation, as measured by the Fanconi D2 protein (FANCD2) monoubiquitination. [beta]-catenin and FANCC nuclear entry is defective in FA mutant cells and in cells depleted of the Fanconi A protein or FANCD2, suggesting that integrity of the FA pathway is required for FANCC nuclear activity. We also report that FANCC with CtBP1 acts as a negative regulator of Dickkopf-1 (DKK1) expression, and that a FA disease-causing mutation in FANCC abrogates this function. Our findings reveal that a defective FA pathway leads to up-regulation of DKK1, a molecule involved in hematopoietic malignancies. www.pnas.org/cgi/doi/10.1073/pnas.1314226111
language: eng
source:
identifier: E-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1314226111
fulltext: fulltext
issn:
  • 10916490
  • 1091-6490
url: Link


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titleThe Fanconi anemia pathway has a dual function in Dickkopf-1 transcriptional repression.
creatorHuard, Caroline C ; Tremblay, Cédric S ; Magron, Audrey ; Lévesque, Georges ; Carreau, Madeleine
contributorHuard, Caroline C (correspondence author) ; Huard, Caroline C (record owner)
ispartofProceedings of the National Academy of Sciences of the United States of America, February 11, 2014, Vol.111(6), pp.2152-2157
identifierE-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1314226111
subjectEnzyme Activation–Genetics ; Fanconi Anemia–Metabolism ; Fanconi Anemia Complementation Group C Protein–Metabolism ; Gene Expression Regulation–Metabolism ; Glycogen Synthase Kinase 3–Genetics ; Glycogen Synthase Kinase 3 Beta–Genetics ; Hek293 Cells–Genetics ; Humans–Genetics ; Intercellular Signaling Peptides and Proteins–Genetics ; Transcription, Genetic–Genetics ; Dkk1 Protein, Human ; Fanconi Anemia Complementation Group C Protein ; Intercellular Signaling Peptides and Proteins ; Gsk3b Protein, Human ; Glycogen Synthase Kinase 3 Beta ; Glycogen Synthase Kinase 3
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descriptionFanconi anemia (FA) is an inherited bone marrow failure syndrome associated with a progressive decline in hematopoietic stem cells, developmental defects, and predisposition to cancer. These various phenotypic features imply a role of FA proteins in molecular events regulating cellular homeostasis. Interestingly, we previously found that the Fanconi C protein (FANCC) interacts with the C-terminal-binding protein-1 (CtBP1) involved in transcriptional regulation. Here we report that FANCC with CtBP1 forms a complex with [beta]-catenin, and that [beta]-catenin activation through glycogen synthase kinase 3[beta] inhibition leads to FANCC nuclear accumulation and FA pathway activation, as measured by the Fanconi D2 protein (FANCD2) monoubiquitination. [beta]-catenin and FANCC nuclear entry is defective in FA mutant cells and in cells depleted of the Fanconi A protein or FANCD2, suggesting that integrity of the FA pathway is required for FANCC nuclear activity. We also report that FANCC with CtBP1 acts as a negative regulator of Dickkopf-1 (DKK1) expression, and that a FA disease-causing mutation in FANCC abrogates this function. Our findings reveal that a defective FA pathway leads to up-regulation of DKK1, a molecule involved in hematopoietic malignancies. www.pnas.org/cgi/doi/10.1073/pnas.1314226111
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