Literature DB >> 28811338

Emerging functions of the Fanconi anemia pathway at a glance.

Rhea Sumpter1, Beth Levine2,3.   

Abstract

Fanconi anemia (FA) is a rare disease, in which homozygous or compound heterozygous inactivating mutations in any of 21 genes lead to genomic instability, early-onset bone marrow failure and increased cancer risk. The FA pathway is essential for DNA damage response (DDR) to DNA interstrand crosslinks. However, proteins of the FA pathway have additional cytoprotective functions that may be independent of DDR. We have shown that many FA proteins participate in the selective autophagy pathway that is required for the destruction of unwanted intracellular constituents. In this Cell Science at a Glance and the accompanying poster, we briefly review the role of the FA pathway in DDR and recent findings that link proteins of the FA pathway to selective autophagy of viruses and mitochondria. Finally, we discuss how perturbations in FA protein-mediated selective autophagy may contribute to inflammatory as well as genotoxic stress.
© 2017. Published by The Company of Biologists Ltd.

Entities:  

Keywords:  DNA damage response; Fanconi anemia; Inflammasome; Mitophagy; Selective autophagy; Virophagy

Mesh:

Substances:

Year:  2017        PMID: 28811338      PMCID: PMC5576063          DOI: 10.1242/jcs.204909

Source DB:  PubMed          Journal:  J Cell Sci        ISSN: 0021-9533            Impact factor:   5.285


  60 in total

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5.  The Fanconi anemia complementation group C gene product: structural evidence of multifunctionality.

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6.  FANCC interacts with Hsp70 to protect hematopoietic cells from IFN-gamma/TNF-alpha-mediated cytotoxicity.

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7.  Fanconi Anemia Proteins Function in Mitophagy and Immunity.

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Journal:  PLoS One       Date:  2018-12-27       Impact factor: 3.240

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Review 9.  Recent advances in understanding hematopoiesis in Fanconi Anemia.

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10.  Fanconi anemia proteins counteract the implementation of the oncogene-induced senescence program.

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