Malignant Pleural Mesothelioma: Present Status and Future Directions

Malignant pleural mesothelioma is a life-threatening neoplasm of the mesothelium, the layer of tissue that covers many organs in the human body. The incidence of this type of neoplasm is gradually ...
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Somatic and Germline BAP1 Mutations in Malignant Mesothelioma

Pp. 193-203 (11)

Andrea Napolitano, Laura Pellegrini, Haining Yang and Michele Carbone

Abstract

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.

Affiliation:

University of Hawaii Cancer Center, 701 Ilalo St #600, 96813, Honolulu, HI, United States.