Journal
JOURNAL OF CELL SCIENCE
Volume 130, Issue 16, Pages 2657-2662Publisher
COMPANY OF BIOLOGISTS LTD
DOI: 10.1242/jcs.204909
Keywords
Selective autophagy; Fanconi anemia; Mitophagy; Inflammasome; Virophagy; DNA damage response
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Funding
- National Institutes of Health [K08 AI099150, U19 AI109725, RO1 CA109618]
- Burroughs Wellcome Fund
- University of Texas Southwestern Medical Center President's Research Council
- Cancer Prevention and Research Institute of Texas [RP120718]
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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 proteinmediated selective autophagy may contribute to inflammatory as well as genotoxic stress.
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