| Literature DB >> 28811338 |
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.Entities:
Keywords: DNA damage response; Fanconi anemia; Inflammasome; Mitophagy; Selective autophagy; Virophagy
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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