What is wrong with Fanconi anemia cells?

Cantor, Sharon B.; Brosh, Robert M.

doi:10.4161/15384101.2014.980633

Cited by 16 publications

(13 citation statements)

References 74 publications

(76 reference statements)

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“…This result suggests that the WRN protein may play an important role in repairing a specific class of DSB in human cells. Fanconi anemia is a blood disorder associated with a genetic defect in a cluster of proteins responsible for DNA repair and results in bone marrow failure [102]. The repair kinetics of radiation-induced DSBs were assessed in primary fibroblasts from Fanconi anemia, non-fanconic anemia bone marrow failure (non-FABMF) and control cell lines based on a gH2AX assay.…”

Section: Fibroblastsmentioning

confidence: 99%

Persistent γH2AX: A promising molecular marker of DNA damage and aging

Siddiqui

François

Fenech

et al. 2015

Mutation Research/Reviews in Mutation Research

167

142

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Section: Fibroblastsmentioning

confidence: 99%

Persistent γH2AX: A promising molecular marker of DNA damage and aging

Siddiqui

François

Fenech

et al. 2015

Mutation Research/Reviews in Mutation Research

167

142

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“…Given the participation of homologous recombination and error-prone TLS in ICL repair, it is possible that MMR proteins might modulate these steps [139]. In addition, it is proposed that crosstalk between FA and MMR pathways is important to regulate repair, and that a FANCJ–MLH1 interaction suppresses MSH2 activity to promote restart at stalled replication forks [140,141]. A p53-independent apoptotic pathway involving the p53-related transcription factor p73 has been described for the MLH1-mediated damage response to cisplatin [142].…”

Section: Oxidative Damage and Dna Adductsmentioning

confidence: 99%

DNA mismatch repair and the DNA damage response

2016

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This review discusses the role of DNA mismatch repair (MMR) in the DNA damage response (DDR) that triggers cell cycle arrest and, in some cases, apoptosis. Although the focus is on findings from mammalian cells, much has been learned from studies in other organisms including bacteria and yeast [1,2]. MMR promotes a DDR mediated by a key signaling kinase, ATM and Rad3-related (ATR), in response to various types of DNA damage including some encountered in widely used chemotherapy regimes. An introduction to the DDR mediated by ATR reveals its immense complexity and highlights the many biological and mechanistic questions that remain. Recent findings and future directions are highlighted.

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“…As has been known for many years, cells from FA patients are highly sensitive to oxidative stress (Joenje et al, 1981) and show increased levels of oxidative DNA damage (Pagano et al, 2005). Consequently, in addition to its identity as a BMF syndrome, FA is recognized as a DNA repair disorder (Cantor and Brosh, Jr., 2014). More broadly, FA displays features symptomatic of accelerated aging ( Figure 1 ), which will be the emphasis of this review article.…”

Section: Aging At the Cellular Levelmentioning

confidence: 99%

Fanconi Anemia: A DNA repair disorder characterized by accelerated decline of the hematopoietic stem cell compartment and other features of aging

Brosh

Bellani

Liu

et al. 2017

Ageing Research Reviews

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Fanconi Anemia (FA) is a rare autosomal genetic disorder characterized by progressive bone marrow failure (BMF), endocrine dysfunction, cancer, and other clinical features commonly associated with normal aging. The anemia stems directly from an accelerated decline of the hematopoietic stem cell compartment. Although FA is a complex heterogeneous disease linked to mutations in 19 currently identified genes, there has been much progress in understanding the molecular pathology involved. FA is broadly considered a DNA repair disorder and the FA gene products, together with other DNA repair factors, have been implicated in interstrand cross-link (ICL) repair. However, in addition to the defective DNA damage response, altered epigenetic regulation, and telomere defects, FA is also marked by elevated levels of inflammatory mediators in circulation, a hallmark of faster decline in not only other hereditary aging disorders but also normal aging. In this review, we offer a perspective of FA as a monogenic accelerated aging disorder, citing the latest evidence for its multi-factorial deficiencies underlying its unique clinical and cellular features.

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What is wrong with Fanconi anemia cells?

Cited by 16 publications

References 74 publications

Persistent γH2AX: A promising molecular marker of DNA damage and aging

Persistent γH2AX: A promising molecular marker of DNA damage and aging

DNA mismatch repair and the DNA damage response

Fanconi Anemia: A DNA repair disorder characterized by accelerated decline of the hematopoietic stem cell compartment and other features of aging

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