| Literature DB >> 27844240 |
Makia J Marafie1, Mohammed Dashti2, Fahd Al-Mulla3.
Abstract
Nijmegen breakage syndrome is an autosomal recessive disorder caused by biallelic mutation in NBN gene. It is characterized by microcephaly, growth retardation, immuno-deficiency and cancer predisposition. The monoallelic carriers of NBN gene are also reported to be at increased risk of developing various types of malignancy. We have investigated an individual with lung cancer from an extended family segregating different types of hereditary cancer over several generations, including lung, breast, ovarian, colon, prostate and renal cancers. By using next generation whole exome sequencing approach, we identified a rare heterozygous frameshift mutation in NBN gene; c.93_94delTG (Ala32HisfsTer4), which is predicted to be pathogenic together with 3 other variants; 2 being in the BRCA1 gene, c.1648A > C (p.Asn550His) and c.536A > G (p.Tyr179Cys), and one in RAD50 gene, c.3539G > A (p.Arg1180Gln). Some of the variants were also found in six out of eight clinically normal relatives, but in different combinations. To our knowledge, this is the first report of NBN gene mutation in an individual with lung cancer in the Arab world. Reporting such findings may aid in variants' risk classification and clinical decision in the future.Entities:
Keywords: Arab; BRCA1; Lung cancer; NBN; Next generation sequencing; Whole-exome sequencing
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Year: 2017 PMID: 27844240 DOI: 10.1007/s10689-016-9954-9
Source DB: PubMed Journal: Fam Cancer ISSN: 1389-9600 Impact factor: 2.375