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MetabolomicsDistinct succinate modifying products are clinically diagnostic and prognostic biomarkers for fumarate hydratase deficient renal cell carcinoma

Germline or somatic loss-of-function of fumarate hydratase (FH) predisposes patients to an aggressive form of renal cell carcinoma (RCC). Since other than tumor resection, there is no effective therapy for FH-deficient RCC, a competent method for early diagnosis and for early detection of recurre...

2022-08-12 | MTBLS3628 | MetaboLights
TranscriptomicsTranscription profiling of human fibriods vs. normal myometrium with mutated or wild type fumarate hydratase (FH) gene reveals distinct expression profiles in fumarate-hydratase-deficient uterine fibroids.
A series of gene expression measurements of normal myometrium and uterine fibroids with mutated or wild-type fumarate hydratase (FH) gene.
ORGANISM(S): Homo sapiens 
2007-10-02 | E-GEOD-2725 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of human uterine fibroids with mutated or wild type fumarate hydratase gene
A series of gene expression measurements of uterine fibroids with mutated or wild-type fumarate hydratase (FH) gene.
ORGANISM(S): Homo sapiens 
2007-08-25 | E-GEOD-2152 | biostudies-arrayexpress
ProteomicsMudPIT analyses of the protein succination after fumarate treatment of whole cell extracts from Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC) fumarate hydratase-deficient (FH-/-) cells
In brief, TCA-precipitated proteins from whole cell extracts treated with fumarate were urea-denatured, reduced, alkylated, and digested with endoproteinase LysC followed by trypsin. The peptide mixtures were loaded onto microcapillary fused silica columns (100um i.d.), packed with C18 reverse phase...
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
2018-03-13 | MSV000082160 | MassIVE
TranscriptomicsTranscription profiling of human uterine fibroids and normal myometrium
Defects in mitochondrial enzymes predispose to severe developmental defects as well as tumorigenesis. Heterozygous germline mutations in the nuclear gene encoding fumarate hydratase (FH), an enzyme catalyzing the hydration of fumarate in the Krebs tricarboxylic acid cycle, cause hereditary leiomyoma...
ORGANISM(S): Homo sapiens 
2007-10-02 | E-GEOD-2724 | biostudies-arrayexpress
ProteomicsFumarate hydratase suppresses glioblastoma progression via a TRIM17-RRN3 proteostatic axis
Emerging evidence indicates that metabolic reprogramming plays a pivotal role in glioblastoma (GBM) progression. Here, we report that the expression of fumarate hydratase (FH) is significantly reduced in GBM patient samples. FH overexpression suppresses the viability, migration, and invasion of GBM ...
ORGANISM(S): Homo sapiens (Human) 
2026-09-14 | PXD067085 | Pride
Human Succination U87 Gbm Lc-ms/ms 
ProteomicsA NOVEL REGULATION OF FUMARATE HYDRATASE ACTIVITY THROUGH HISTONE DEACETYLASE 6 BINDING
Introduction: Altered metabolism is a key hallmark of tumour cells (Pavlova & Thompson, 2016) and has long been associated with relapse and resistance to treatment (Gonçalves et al., 2021; Lv et al., 2022). Recently, we identified a new role for histone deacetylase 6 (HDAC6) in the regulation of gl...
ORGANISM(S): Homo sapiens (Human) 
2025-08-04 | PXD057865 | Pride
Fumarate hydratase Regulation Histone deacetylase 6. 
ProteomicsA chemoproteomic portrait of the oncometabolite fumarate
Hereditary cancer disorders often provide an important window into novel mechanisms supporting tumor growth and survival. Understanding these mechanisms and developing biomarkers to identify their presence thus represents a vital goal. Towards this goal, here we report a chemoproteomic map of the co...
ORGANISM(S): Homo sapiens (Human) 
2018-11-27 | PXD009378 | Pride
Lc-msms Uok262 Fumarate Hereditary leiomyomatosis and renal cell carcinoma (hlrcc) Succination Fumarate hydratase (fh) Cysteine reactivity 
TranscriptomicsThe role of HIF in FH-associated renal neoplasia
Fumarate hydratase (FH) mutations predispose to renal cysts cancer. These cancers overexpress hypoxia-inducible factor-alpha (Hif-1a). We have generated a conditional Fh1 (mouse FH) knockout mice that develop renal cysts and overexpress Hif-1a. In order to identify the contribution of Hif-1a to cyst...
ORGANISM(S): Mus musculus 
2011-10-23 | E-GEOD-29988 | biostudies-arrayexpress
TranscriptomicsAn antioxidant response phenotype is shared between hereditary and sporadic type 2 papillary renal cell carcinoma
Fumarate hydratase (FH) mutation causes hereditary type 2 papillary renal cell carcinoma (HLRCC, Hereditary Leiomyomatosis and Renal Cell Cancer (MM ID # 605839)). The main effect of FH mutation is fumarate accumulation. The current paradigm posits that the main consequence of fumarate accumulation ...
ORGANISM(S): Homo sapiens 
2011-10-17 | E-GEOD-26574 | biostudies-arrayexpress
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