Ontology highlight
ABSTRACT:
SUBMITTER: Sourbier C
PROVIDER: S-EPMC6895110 | biostudies-literature | 2019 Dec
REPOSITORIES: biostudies-literature
Sourbier Carole C Ricketts Christopher J CJ Liao Pei-Jyun PJ Matsumoto Shingo S Wei Darmood D Lang Martin M Railkar Reema R Yang Youfeng Y Wei Ming-Hui MH Agarwal Piyush P Krishna Murali M Mitchell James B JB Trepel Jane B JB Neckers Len L Linehan W Marston WM
Scientific reports 20191205 1
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is characterized by germline mutations of the FH gene that encodes for the TCA cycle enzyme, fumarate hydratase. HLRCC patients are at risk for the development of an aggressive form of type 2 papillary renal cell carcinoma. By studying the mechanism of action of marizomib, a proteasome inhibitor able to cross the blood-brain barrier, we found that it modulates the metabolism of HLRCC cells. Marizomib decreased glycolysis in vitro and in ...[more]