Mechanisms of vertebrate DNA interstrand cross-link repair

DR Semlow, JC Walter - Annual review of biochemistry, 2021 - annualreviews.org
DNA interstrand cross-links (ICLs) covalently connect the two strands of the double helix and
are extremely cytotoxic. Defective ICL repair causes the bone marrow failure and cancer …

Fanconi anemia: current insights regarding epidemiology, cancer, and DNA repair

JD Peake, E Noguchi - Human genetics, 2022 - Springer
Fanconi anemia is a genetic disorder that is characterized by bone marrow failure, as well
as a predisposition to malignancies including leukemia and squamous cell carcinoma …

Recent discoveries in the molecular pathogenesis of the inherited bone marrow failure syndrome Fanconi anemia

NE Mamrak, A Shimamura, NG Howlett - Blood reviews, 2017 - Elsevier
Fanconi anemia (FA) is a rare autosomal and X-linked genetic disease characterized by
congenital abnormalities, progressive bone marrow failure (BMF), and increased cancer risk …

Cellular response to DNA interstrand crosslinks: the Fanconi anemia pathway

D Lopez-Martinez, CC Liang, MA Cohn - Cellular and molecular life …, 2016 - Springer
Interstrand crosslinks (ICLs) are a highly toxic form of DNA damage. ICLs can interfere with
vital biological processes requiring separation of the two DNA strands, such as replication …

Mechanism of ubiquitination and deubiquitination in the Fanconi anemia pathway

S van Twest, VJ Murphy, C Hodson, W Tan, P Swuec… - Molecular cell, 2017 - cell.com
Monoubiquitination and deubiquitination of FANCD2: FANCI heterodimer is central to DNA
repair in a pathway that is defective in the cancer predisposition syndrome Fanconi anemia …

What is the DNA repair defect underlying Fanconi anemia?

JP Duxin, JC Walter - Current opinion in cell biology, 2015 - Elsevier
Fanconi anemia (FA) is a rare human genetic disease characterized by bone marrow failure,
cancer predisposition, and genomic instability. It has been known for many years that FA …

Structure of the Fanconi anaemia monoubiquitin ligase complex

S Shakeel, E Rajendra, P Alcón, F O'Reilly, DS Chorev… - Nature, 2019 - nature.com
The Fanconi anaemia (FA) pathway repairs DNA damage caused by endogenous and
chemotherapy-induced DNA crosslinks, and responds to replication stress,. Genetic …

[HTML][HTML] Fanconi anemia

PA Mehta, C Ebens - 2021 - europepmc.org
Fanconi anemia (FA) is characterized by physical abnormalities, bone marrow failure, and
increased risk for malignancy. Physical abnormalities, present in approximately 75% of …

FANCA promotes DNA double-strand break repair by catalyzing single-strand annealing and strand exchange

A Benitez, W Liu, A Palovcak, G Wang, J Moon, K An… - Molecular cell, 2018 - cell.com
FANCA is a component of the Fanconi anemia (FA) core complex that activates DNA
interstrand crosslink repair by monoubiquitination of FANCD2. Here, we report that purified …

Binding of FANCI-FANCD2 complex to RNA and R-loops stimulates robust FANCD2 monoubiquitination

Z Liang, F Liang, Y Teng, X Chen, J Liu, S Longerich… - Cell reports, 2019 - cell.com
Fanconi anemia (FA) is characterized by developmental abnormalities, bone marrow failure,
and cancer predisposition. FA cells are hypersensitive to DNA replicative stress and …