Pathology · Neoplasia (Classification, Carcinogenesis, Tumor Markers, Paraneoplastic)

The Warburg effect in cancer cells refers to preferential aerobic glycolysis over oxidative phosphorylation even in the presence of adequate oxygen. The oncometabolite that most directly promotes HIF-1alpha stabilization in the normoxic tumor microenvironment of IDH1-mutant gliomas is:

  • A Succinate, which inhibits prolyl hydroxylase domain (PHD) enzymes
  • B Fumarate, which alkylates and inhibits PHD2 via succination
  • C 2-hydroxyglutarate (2-HG), which competitively inhibits alpha-ketoglutarate-dependent dioxygenases including PHDs
  • D Lactate, which acidifies the cytosol and prevents VHL-mediated HIF-1alpha ubiquitination
Correct answer: C. 2-hydroxyglutarate (2-HG), which competitively inhibits alpha-ketoglutarate-dependent dioxygenases including PHDs

Explanation

IDH1/2 mutations produce the oncometabolite R-2-hydroxyglutarate (2-HG) from alpha-ketoglutarate. 2-HG competitively inhibits alpha-KG-dependent dioxygenases, including TET2, histone demethylases, and prolyl hydroxylase domain (PHD) enzymes. PHD inhibition prevents hydroxylation of HIF-1alpha, which is required for VHL-mediated proteasomal degradation, leading to normoxic HIF-1alpha stabilization and a pseudo-hypoxic transcriptional program. Succinate accumulation (SDH mutations) and fumarate (FH mutations) similarly inhibit PHDs, but these are not the mechanism in IDH-mutant tumors. Lactate acidification is not the primary mechanism for HIF stabilization.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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