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Trends Biochem Sci. 2019 Jan 10. pii: S0968-0004(18)30218-4. doi: 10.1016/j.tibs.2018.10.008. [Epub ahead of print]

The Tumor Suppressor PALB2: Inside Out.

Author information

1
CHU de Québec Research Center, Oncology Division, 9 McMahon, Québec City, QC, G1R 3S3, Canada; CHU de Québec Research Center, Endocrinology and Nephrology Division, 2705 Bld Laurier, Québec City, QC, G1V 4G2, Canada; Department of Molecular Biology, Medical Biochemistry and Pathology, Laval University Cancer Research Center, Québec City, QC, G1V 0A6, Canada.
2
CHU de Québec Research Center, Oncology Division, 9 McMahon, Québec City, QC, G1R 3S3, Canada; Department of Molecular Biology, Medical Biochemistry and Pathology, Laval University Cancer Research Center, Québec City, QC, G1V 0A6, Canada.
3
CHU de Québec Research Center, Oncology Division, 9 McMahon, Québec City, QC, G1R 3S3, Canada; CHU de Québec Research Center, Endocrinology and Nephrology Division, 2705 Bld Laurier, Québec City, QC, G1V 4G2, Canada.
4
CHU de Québec Research Center, Oncology Division, 9 McMahon, Québec City, QC, G1R 3S3, Canada; Department of Molecular Biology, Medical Biochemistry and Pathology, Laval University Cancer Research Center, Québec City, QC, G1V 0A6, Canada. Electronic address: Jean-Yves.Masson@crchudequebec.ulaval.ca.

Abstract

Partner and Localizer of BRCA2 (PALB2) has emerged as an important and versatile player in genome integrity maintenance. Biallelic mutations in PALB2 cause Fanconi anemia (FA) subtype FA-N, whereas monoallelic mutations predispose to breast, and pancreatic familial cancers. Herein, we review recent developments in our understanding of the mechanisms of regulation of the tumor suppressor PALB2 and its functional domains. Regulation of PALB2 functions in DNA damage response and repair occurs on multiple levels, including homodimerization, phosphorylation, and ubiquitylation. With a molecular emphasis, we present PALB2-associated cancer mutations and their detailed analysis by functional assays.

KEYWORDS:

DNA double-strand break repair; Fanconi anemia; cancer; homologous recombination; tumor suppressor

PMID:
30638972
DOI:
10.1016/j.tibs.2018.10.008

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