Interstrand cross-links (ICLs) block replication and transcription and thus are highly cytotoxic. In higher eukaryotes, ICLs processing involves the Fanconi anemia (FA) pathway and homologous recombination. Stalled replication forks activate the eight-subunit FA core complex, which ubiquitylates FANCD2-FANCI. Once it is posttranslationally modified...
Interstrand cross-links (ICLs) block replication and transcription and thus are highly cytotoxic. In higher eukaryotes, ICLs processing involves the Fanconi anemia (FA) pathway and homologous recombination. Stalled replication forks activate the eight-subunit FA core complex, which ubiquitylates FANCD2-FANCI. Once it is posttranslationally modified...
Interstrand cross-links (ICLs) block replication and transcription and thus are highly cytotoxic. In higher eukaryotes, ICLs processing involves the Fanconi anemia (FA) pathway and homologous recombination. Stalled replication forks activate the eight-subunit FA core complex, which ubiquitylates FANCD2-FANCI. Once it is posttranslationally modified...
Published in Methods in cell biology
Fanconi anemia (FA) is a human disease of bone marrow failure, leukemia, squamous cell carcinoma, and developmental anomalies, including hypogonadism and infertility. Bone marrow transplants improve hematopoietic phenotypes but do not prevent other cancers. FA arises from mutation in any of the 15 FANC genes that cooperate to repair double stranded...
Published in ORL; journal for oto-rhino-laryngology and its related specialties
Fanconi anemia (FA) and dyskeratosis congenita (DC) are rare inherited syndromes that cause head and neck squamous cell cancer (HNSCC). Prior studies of inherited forms of cancer have been extremely important in elucidating tumor suppressor genes inactivated in sporadic tumors. Here, we studied whether sporadic tumors have epigenetic silencing of t...
Published in Comprehensive Toxicology
Cells are permanently exposed to genotoxic insults from internal metabolism and from exposures to chemicals and environmental agents giving rise to lesions in deoxyribonucleic acid (DNA). If not repaired, these may lead to cell death or to mutations, chromosomal aberrations, and initiation of carcinogenesis. During evolution, efficient multienzymat...
Published in Comprehensive Toxicology
The majority of human cancers result from exposure to environmental carcinogens. Epidemiologic studies, as well as studies in animal models of cancer, have demonstrated a wide range of responses to carcinogen exposure and it is now clear that common allelic variants within the genome are responsible for this interindividual variation. Identificatio...
Published in Cytogenetic and Genome Research
The Bloom protein (BLM) and Topoisomerase IIIα are found in association with proteins of the Fanconi anemia (FA) pathway, a disorder manifesting increased cellular sensitivity to DNA crosslinking agents. In order to determine if the association reflects a functional interaction for the maintenance of genome stability, we have analyzed the effects o...
Published in Mutation Research/Fundamental and Molecular Mechanisms of Mutagenesis
Abstract Fanconi anemia (FA) is a rare autosomal recessive or X-linked disorder characterized by aplastic anemia, cancer susceptibility and cellular sensitivity to DNA-crosslinking agents. Eight FA proteins (FANCA, -B, -C, -E, -F, -G, -L and -M) and three non-FA proteins (FAAP100, FAAP24 and HES1) form the FA nuclear core complex that is required f...
La préservation d’un stock de cellules souches fonctionnelles est indispensable pour le maintien de nombreux tissus chez l’adulte. Les cellules souches se multiplient pour s’auto-renouveller et entrent en différenciation donnant naissance à des progéniteurs puis à des cellules matures. Récemment, la possibilité pour les progéniteurs de se reprogram...
