{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Panarsky R"],"funding":["NIDDK NIH HHS","National Cancer Institute"],"pagination":["7"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC7397616"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["8"],"pubmed_abstract":["Background
The loss-of-function mutation of fumarate hydratase (FH) is a driver of hereditary leiomyomatosis and renal cell carcinoma (HLRCC). Fumarate accumulation results in activation of stress-related mechanisms leading to upregulation of cell survival-related genes. To better understand how cells compensate for the loss of FH in HLRCC, we determined the amino acid nutrient requirements of the FH-deficient UOK262 cell line (UOK262) and its FH-repleted control (UOK262WT).Methods
We determined growth rates and survival of cell lines in response to amino acid depletion and supplementation. RNAseq was used to determine the transcription changes contingent on Asn and Gln supplementation, which was further followed with stable isotope resolved metabolomics (SIRM) using both ["],"journal":["Cancer & metabolism"],"pubmed_title":["Fumarate hydratase-deficient renal cell carcinoma cells respond to asparagine by activation of the unfolded protein response and stimulation of the hexosamine biosynthetic pathway."],"pmcid":["PMC7397616"],"funding_grant_id":["U24 DK097215","1U24DK097215-01A1, 3R01ES022191-04S1, and P30CA177558"],"pubmed_authors":["Crooks DR","Linehan WM","Yang Y","Moscow JA","Panarsky R","Fan TW","Lane AN","Cassel TA"],"additional_accession":[]},"is_claimable":false,"name":"Fumarate hydratase-deficient renal cell carcinoma cells respond to asparagine by activation of the unfolded protein response and stimulation of the hexosamine biosynthetic pathway.","description":"Background
The loss-of-function mutation of fumarate hydratase (FH) is a driver of hereditary leiomyomatosis and renal cell carcinoma (HLRCC). Fumarate accumulation results in activation of stress-related mechanisms leading to upregulation of cell survival-related genes. To better understand how cells compensate for the loss of FH in HLRCC, we determined the amino acid nutrient requirements of the FH-deficient UOK262 cell line (UOK262) and its FH-repleted control (UOK262WT).Methods
We determined growth rates and survival of cell lines in response to amino acid depletion and supplementation. RNAseq was used to determine the transcription changes contingent on Asn and Gln supplementation, which was further followed with stable isotope resolved metabolomics (SIRM) using both [","dates":{"release":"2020-01-01T00:00:00Z","publication":"2020","modification":"2025-05-29T22:10:32.529Z","creation":"2025-05-29T22:10:32.529Z"},"accession":"S-EPMC7397616","cross_references":{"pubmed":["32774853"],"doi":["10.1186/s40170-020-00214-9"]}}