HNRNPC-mediated RNA processing promotes VHL-associated excessive vasculogenesis in von Hippel-Lindau syndrome revealed from patient-derived blood vessel models

HNRNPC stabilization in VHL-inactivated, patient-derived vascular cells drives excessive blood vessel formation in a human iPSC model of von Hippel-Lindau syndrome.

• Why it matters: VHLS vasculogenesis contributes to hemangioblastomas and RCC, yet HIF2A-targeted therapies show limited response, creating a need for HIF2A-independent mechanisms and targets.
• What they did: The study built a human iPSC-based VHLS vascular platform using patient iPSCs and VHL-edited isogenic lines, then compared vessel formation and tested RBPs, focusing on HNRNPC stabilization.
• The result: HNRNPC stabilization was critical for dysregulated vascularization in vivo, enabling an iPSC-derived system to dissect VHLS biology and nominate HNRNPC as a potential therapeutic target via an HIF2A-independent pathway.

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