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Citations to this article

Cytoplasmic localization of FAC is essential for the correction of a prerepair defect in Fanconi anemia group C cells.
H Youssoufian
H Youssoufian
Published May 1, 1996
Citation Information: J Clin Invest. 1996;97(9):2003-2010. https://doi.org/10.1172/JCI118635.
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Cytoplasmic localization of FAC is essential for the correction of a prerepair defect in Fanconi anemia group C cells.

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Abstract

Mutations in the gene defective in Fanconi anemia complementation group C, FAC, are responsible for a subset of Fanconi anemia, a group of autosomal recessive disorders characterized by chromosomal instability, hypersensitivity to cross-linking agents, and cancer susceptibility. Although abnormalities in DNA repair have been suspected, localization of the FAC gene product to the cytoplasm has cast doubt on such a mechanism. Monitoring of interstrand DNA cross-linking shows that the predominant defect in group C cells is in the initial induction of cross-links, not in repair synthesis. Both the cross-linking defect and the enhanced cytotoxicity of cross-linkers on Fanconi anemia group C cells are corrected completely by cytoplasmic isoforms of the FAC protein, but not by an isoform targeted to the nucleus. The ability of FAC to correct these phenotypic abnormalities reaches a maximum threshold despite overexpression leading to higher levels of cytosolic protein. These results demonstrate that cytoplasmic localization is essential for the intracellular activity of the FAC protein. It is proposed that this activity is coupled to a cytoplasmic defense mechanism against a specific class of genotoxic agents.

Authors

H Youssoufian

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Total citations by year

Year: 2024 2018 2012 2004 2003 2002 2001 2000 1999 1998 1997 Total
Citations: 1 1 1 3 4 2 7 6 7 9 6 47
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Citations to this article (47)

Title and authors Publication Year
HROB Is Implicated in DNA Replication
Kutz J, Schmietendorf H, Rahman SA, Opel F, Pospiech H
Genes 2024
Fanconi anemia complementation group C protection against oxidative stress‑induced β‑cell apoptosis
S Kulanuwat, P Jungtrakoon, W Tangjittipokin, PT Yenchitsomanus, N Plengvidhya
Molecular medicine reports 2018
Fanconi Anemia Proteins and Their Interacting Partners: A Molecular Puzzle
T Kaddar, M Carreau
Anemia 2012
Genetic Predisposition to Cancer, 2Ed
J Birch
Genetic Predisposition to Cancer, 2Ed 2004
Oxidant Hypersensitivity of Fanconi Anemia Type C-deficient Cells Is Dependent on a Redox-regulated Apoptotic Pathway
MR Saadatzadeh, K Bijangi-Vishehsaraei, P Hong, H Bergmann, LS Haneline
The Journal of biological chemistry 2004
The Fanconi Anemia Core Complex Forms Four Complexes of Different Sizes in Different Subcellular Compartments
A Thomashevski, AA High, M Drozd, J Shabanowitz, DF Hunt, PA Grant, GM Kupfer
The Journal of biological chemistry 2004
Increased sensitivity of Fancc-deficient hematopoietic cells to nitric oxide and evidence that this species mediates growth inhibition by cytokines
S Hadjur, FR Jirik
Blood 2003
Chromosomal Instability and Aging: Basic Science and Clinical Implications
D Schindler, M Wagner, H Hoehn, MT Gross, A Sobeck
Chromosomal Instability and Aging: Basic Science and Clinical Implications 2003
Lymphoma development in Bax transgenic mice is inhibited by Bcl-2 and associated with chromosomal instability
JJ Luke, CI van de Wetering, CM Knudson
Cell Death and Differentiation 2003
Fanconi anemia protein complex: mapping protein interactions in the yeast 2- and 3-hybrid systems
SM Gordon, M Buchwald
Blood 2003
Fanconi anemia group A and C double-mutant mice
M Noll, KP Battaile, R Bateman, TP Lax, K Rathbun, C Reifsteck, G Bagby, M Finegold, S Olson, M Grompe
Experimental Hematology 2002
Clinically Relevant Resistance in Cancer Chemotherapy
B Andersson, D Murray
2002
Correction of cross-linker sensitivity of Fanconi anemia group F cells by CD33-mediated protein transfer
RK Holmes, K Harutyunyan, M Shah, H Joenje, H Youssoufian
Blood 2001
Positional Cloning of a Novel Fanconi Anemia Gene, FANCD2
C Timmers, T Taniguchi, J Hejna, C Reifsteck, L Lucas, D Bruun, M Thayer, B Cox, S Olson, AD D'Andrea, R Moses, M Grompe
Molecular Cell 2001
Foci on Fanconi
M Hoatlin, T Reuter
Trends in Molecular Medicine 2001
The Fanconi anemia group C gene product
S Fagerlie, MW Lensch, Q Pang, GC Bagby
Experimental Hematology 2001
Functional Analysis of the Putative Peroxidase Domain of FANCA, the Fanconi Anemia Complementation Group A Protein
J Ren, H Youssoufian
Molecular Genetics and Metabolism 2001
Fanconi anemia group C protein prevents apoptosis in hematopoietic cells through redox regulation of GSTP1
RC Cumming, J Lightfoot, K Beard, H Youssoufian, PJ O'Brien, M Buchwald
Nature Medicine 2001
Functional correction of FA-C cells withFANCC suppresses the expression of interferon γ–inducible genes
SR Fagerlie, J Diaz, TA Christianson, K McCartan, W Keeble, GR Faulkner, GC Bagby
Blood 2001
Do Fanconi anemia genes control cell response to cross-linking agents by modulating cytochrome P-450 reductase activity?
FA Kruyt, H Youssoufian
Drug Resistance Updates 2000
DNA replication is required To elicit cellular responses to psoralen-induced DNA interstrand cross-links
YM Akkari, RL Bateman, CA Reifsteck, SB Olson, M Grompe
Molecular and cellular biology 2000
Investigation of Fanconi Anemia Protein Interactions by Yeast Two-Hybrid Analysis
PA Huber, AL Medhurst, H Youssoufian, CG Mathew
Biochemical and Biophysical Research Communications 2000
Current Clinical Practice: Advances in the Genetics and Biology of Fanconi Anaemia
AJ Tipping, CG Mathew
Hematology 2000
Posttranscriptional cell cycle–dependent regulation of human FANCC expression
MC Heinrich, KV Silvey, S Stone, AJ Zigler, DJ Griffith, M Montalto, L Chai, Y Zhi, ME Hoatlin
Blood 2000
Interferon-γ–induced apoptotic responses of Fanconi anemia group C hematopoietic progenitor cells involve caspase 8–dependent activation of caspase 3 family members
RK Rathbun, TA Christianson, GR Faulkner, G Jones, W Keeble, M O'Dwyer, GC Bagby
Blood 2000
The Fanconi anemia group C gene product modulates apoptotic responses to tumor necrosis factor-α and Fas ligand but does not suppress expression of receptors of the tumor necrosis factor receptor superfamily
PS Koh, GC Hughes, GR Faulkner, WW Keeble, GC Bagby
Experimental Hematology 1999
Localization of the bloom syndrome helicase to punctate nuclear structures and the nuclear matrix and regulation during the cell cycle: Comparison with the werner's syndrome helicase
V Gharibyan, H Youssoufian
Molecular Carcinogenesis 1999
Interstrand cross-links induce DNA synthesis in damaged and undamaged plasmids in mammalian cell extracts
L Li, CA Peterson, X Lu, P Wei, RJ Legerski
Molecular and cellular biology 1999
Resistance to Mitomycin C Requires Direct Interaction between the Fanconi Anemia Proteins FANCA and FANCG in the Nucleus through an Arginine-rich Domain
FA Kruyt, F Abou-Zahr, H Mok, H Youssoufian
The Journal of biological chemistry 1999
Expression of the Fanconi Anemia Group A Gene (Fanca) During Mouse Embryogenesis
R Abu-Issa, G Eichele, H Youssoufian
Blood 1999
Loss of FancC Function Results in Decreased Hematopoietic Stem Cell Repopulating Ability
LS Haneline, TA Gobbett, R Ramani, M Carreau, M Buchwald, MC Yoder, DW Clapp
Blood 1999
Protein Replacement by Receptor-Mediated Endocytosis Corrects the Sensitivity of Fanconi Anemia Group C Cells to Mitomycin C
H Youssoufian, FA Kruyt, X Li
Blood 1999
Is Fanconi anemia caused by a defect in the processing of DNA damage?
M Buchwald, E Moustacchi
Mutation Research/DNA Repair 1998
Fanconi's anemia: what have we learned from the genes so far?
MC PhD, MB PhD
Molecular Medicine Today 1998
MOLECULAR GENETICS AND FANCONI ANAEMIA: NEW INSIGHTS INTO OLD PROBLEMS
Clarke, Marsh, Gordon-Smith, Rutherford
British Journal of Haematology 1998
Abnormal microsomal detoxification implicated in Fanconi anemia group C by interaction of the FAC protein with NADPH cytochrome P450 reductase
FA Kruyt, T Hoshino, JM Liu, P Joseph, AK Jaiswal, H Youssoufian
Blood 1998
Congenital disorders sharing oxidative stress and cancer proneness as phenotypic hallmarks: prospects for joint research in pharmacology
G Pagano, LG Korkina, UT Brunk, L Chessa, P Degan, D Principe, FJ Kelly, W Malorni, F Pallardó, C Pasquier, I Scovassi, A Zatterale, C Franceschi
Medical Hypotheses 1998
The Fanconi Anemia Group C Gene Product Is Located in Both the Nucleus and Cytoplasm of Human Cells
ME Hoatlin, TA Christianson, WW Keeble, AT Hammond, Y Zhi, MC Heinrich, PA Tower, GC Bagby
Blood 1998
The Fanconi Anemia Proteins FAA and FAC Function in Different Cellular Compartments to Protect Against Cross-Linking Agent Cytotoxicity
FA Kruyt, H Youssoufian
Blood 1998
Molecular Chaperone GRP94 Binds to the Fanconi Anemia Group C Protein and Regulates Its Intracellular Expression
T Hoshino, J Wang, MP Devetten, N Iwata, S Kajigaya, RJ Wise, JM Liu, H Youssoufian
Blood 1998
DNA Cross-Linker–Induced G2/M Arrest in Group C Fanconi Anemia Lymphoblasts Reflects Normal Checkpoint Function
MC Heinrich, ME Hoatlin, AJ Zigler, KV Silvey, AC Bakke, WW Keeble, Y Zhi, CA Reifsteck, M Grompe, MG Brown, RE Magenis, SB Olson, GC Bagby
Blood 1998
MxA overexpression reveals a common genetic link in four Fanconi anemia complementation groups
Y Li, H Youssoufian
Journal of Clinical Investigation 1997
Fanconi anemia group A and D cell lines respond normally to inhibitors of cell cycle regulation
P Johnstone, C Reifsteck, S Kohler, P Worland, S Olson, RE Moses
Somatic Cell and Molecular Genetics 1997
Fanconi Anemia: Genetic Testing in Ashkenazi Jews
AD Auerbach
Genetic Testing 1997
Inactivation of the Fanconi Anemia Group C Gene Augments Interferon-γ–Induced Apoptotic Responses in Hematopoietic Cells
RK Rathbun, GR Faulkner, MH Ostroski, TA Christianson, G Hughes, G Jones, R Cahn, R Maziarz, G Royle, W Keeble, MC Heinrich, M Grompe, PA Tower, GC Bagby
Blood 1997
Cytoplasmic Localization of a Functionally Active Fanconi Anemia Group A–Green Fluorescent Protein Chimera in Human 293 Cells
FA Kruyt, Q Waisfisz, LM Dijkmans, MA Hermsen, H Youssoufian, F Arwert, H Joenje
Blood 1997
Phenotypic Consequences of Mutations in the Fanconi Anemia FAC Gene: An International Fanconi Anemia Registry Study
AP Gillio, PC Verlander, SD Batish, PF Giampietro, AD Auerbach
Blood 1997

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