Role of germline aberrations affecting CTNNA1, MAP3K6 and MYD88 in gastric cancer susceptibility

J Med Genet. 2018 Oct;55(10):669-674. doi: 10.1136/jmedgenet-2017-104962. Epub 2018 Jan 12.

Abstract

Background: In approximately 10% of all gastric cancer (GC) cases, a heritable cause is suspected. A subset of these cases have a causative germline CDH1 mutation; however, in most cases the cause remains unknown. Our objective was to assess to what extent these remaining cases may be explained by germline mutations in the novel candidate GC predisposing genes CTNNA1, MAP3K6 or MYD88.

Methods: We sequenced a large cohort of unexplained young and/or familial patients with GC (n=286) without a CDH1germline mutation for germline variants affecting CTNNA1, MAP3K6 and MYD88 using a targeted next-generation sequencing approach based on single-molecule molecular inversion probes.

Results: Predicted deleterious germline variants were not encountered in MYD88, but recurrently observed in CTNNA1 (n=2) and MAP3K6 (n=3) in our cohort of patients with GC. In contrast to deleterious variants in CTNNA1, deleterious variants in MAP3K6 also occur frequently in the general population.

Conclusions: Based on our results MAP3K6 should no longer be considered a GC predisposition gene, whereas deleterious CTNNA1 variants are confirmed as an infrequent cause of GC susceptibility. Biallelic MYD88 germline mutations are at most a very rare cause of GC susceptibility as no additional cases were identified.

Keywords: cancer: gastric; ctnna1 – map3k6 – myd88; heritability; next generation sequencing.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antigens, CD / genetics*
  • Cadherins / genetics*
  • Cohort Studies
  • Europe
  • Female
  • Genetic Predisposition to Disease
  • Germ-Line Mutation
  • High-Throughput Nucleotide Sequencing
  • Humans
  • MAP Kinase Kinase Kinases / genetics*
  • Male
  • Middle Aged
  • Myeloid Differentiation Factor 88 / genetics*
  • Sequence Analysis, DNA
  • Stomach Neoplasms / genetics*
  • Young Adult
  • alpha Catenin / genetics*

Substances

  • Antigens, CD
  • CDH1 protein, human
  • CTNNA1 protein, human
  • Cadherins
  • MYD88 protein, human
  • Myeloid Differentiation Factor 88
  • alpha Catenin
  • MAP Kinase Kinase Kinases
  • MAP kinase kinase kinase 6