BRCA1-associated protein 1 (BAP1) has recently emerged as a novel
important tumor suppressor gene in malignant mesothelioma. BAP1 is a nuclear
deubiquitinating enzyme with several critical biological functions, including regulation
of gene transcription, DNA damage response and chromosome stability. Somatic BAP1
mutations are found in about 60% of mesotheliomas, and germline BAP1 mutations
have been reported to increase the incidence of malignant mesothelioma and to cause a
novel hereditary cancer predisposition syndrome characterized also by higher incidence
of several other cancers. In this chapter, we reviewed all the current evidences on the
biology of BAP1 and on the clinical implications of its somatic and germline mutations
in regards to malignant mesothelioma.
Keywords: Asbestos, BAP1, genetics, germline, malignant mesothelioma,
somatic.
