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  • Fumarate hydratase (FH) and cancer: a paradigm of oncometabolism
    In this review, we will focus on the recent discoveries on the role of FH loss and consequent fumarate accumulation in oncogenesis Moreover, we will discuss the future directions to improve our understanding of FH loss in cancer and potential therapeutic interventions to treat FH-deficient tumours Fumarate hydratase loss and cancer
  • Implications of Fumarate Hydratase Deficiency (FHD) and Cancer Risk: A . . .
    Fumarate hydratase deficiency (FHD) represents a significant challenge in gynecologic oncology due to its association with an increased risk of hereditary leiomyomatosis and renal cell carcinoma
  • Fumarate Hydratase-Deficient Renal Cell Carcinoma
    Germline pathogenic alterations of the FH (fumerate hydratase) gene are associated with hereditary leiomyomatosis and renal cell carcinoma associated renal cancer These patients present with clinically aggressive renal tumors as well as uterine and cutaneous leiomyomas
  • Pathology Outlines - FH deficient
    Terminology HLRCC syndrome has germline mutations of fumarate hydratase gene, conferring an increased risk of uterine and cutaneous leiomyomata as well as renal cancer The associated renal cell carcinoma is termed HLRCC associated renal cancer Also called FH deficient
  • Fumarate hydratase (FH) and cancer: a paradigm of oncometabolism
    Fumarate hydratase (FH) is an enzyme of the Tricarboxylic Acid (TCA) cycle whose mutations lead to hereditary and sporadic forms of cancer
  • Fumarate hydratase deficiency and cancer: activation of hypoxia . . .
    In this issue of Cancer Cell, Pollard and colleagues describe a genetic mouse model of FH deficiency that recapitulates aspects of the human disease, including HIF activation and renal cysts, enabling further insights into this unusual cancer syndrome
  • Fumarate hydratase-deficient renal cell carcinoma
    June 14, 2025 Fumarate hydratase-deficient renal cell carcinoma (often called FH-deficient RCC) is a rare type of kidney cancer It develops due to mutations (changes) in a gene called fumarate hydratase (FH) FH is important for normal cell metabolism Changes in this gene lead to the formation of a tumour with specific microscopic features
  • Different Fumarate Hydratase Gene Variants Are Associated With Distinct . . .
    Do fumarate hydratase–deficiency (FH-d) pathogenic variants (PVs) cause autosomal dominant fumarate hydratase (FH) tumor predisposition syndrome or hereditary leiomyomatosis and renal cell carcinoma?
  • Fumarate hydratase in cancer: A multifaceted tumour suppressor
    The finding that metabolic enzymes such as fumarate hydratase (FH), succinate dehydrogenase (SDH) and isocitrate dehydrogenase (IDH), when mutated, cause cancer suggested that metabolic dysregulation is not only a consequence of oncogenic transformation but that it can act as cancer driver





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