New strategies in pleural mesothelioma: BAP1 and NF2 as novel targets for therapeutic development and risk assessment

Clin Cancer Res. 2012 Sep 1;18(17):4485-90. doi: 10.1158/1078-0432.CCR-11-2375. Epub 2012 Jul 23.

Abstract

Malignant pleural mesothelioma (MPM) is a highly lethal cancer with limited therapeutic options. Recent work has focused on the frequent somatic inactivation of two tumor suppressor genes in MPM-NF2 (Neurofibromatosis type 2) and the recently identified BAP1 (BRCA associated protein 1). In addition, germline mutations in BAP1 have been identified that define a new familial cancer syndrome, which includes MPM, ocular melanoma, and other cancers. These recent advances may allow screening of high-risk individuals and the development of new therapies that target key pathways in MPM.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Genetic Predisposition to Disease
  • Germ-Line Mutation
  • Humans
  • Mesothelioma* / genetics
  • Mesothelioma* / metabolism
  • Mesothelioma* / therapy
  • Molecular Targeted Therapy
  • Neurofibromatosis 2* / genetics
  • Neurofibromatosis 2* / metabolism
  • Risk Assessment
  • Tumor Suppressor Proteins* / genetics
  • Tumor Suppressor Proteins* / metabolism
  • Ubiquitin Thiolesterase* / genetics
  • Ubiquitin Thiolesterase* / metabolism

Substances

  • BAP1 protein, human
  • Tumor Suppressor Proteins
  • Ubiquitin Thiolesterase