Profilin-1 phosphorylation directs angiocrine expression and glioblastoma progression through HIF-1α accumulation

Nat Cell Biol. 2014 May;16(5):445-56. doi: 10.1038/ncb2954. Epub 2014 Apr 20.

Abstract

The tumour vascular microenvironment supports tumorigenesis not only by supplying oxygen and diffusible nutrients but also by secreting soluble factors that promote tumorigenesis. Here we identify a feedforward mechanism in which endothelial cells (ECs), in response to tumour-derived mediators, release angiocrines driving aberrant vascularization and glioblastoma multiforme (GBM) progression through a hypoxia-independent induction of hypoxia-inducible factor (HIF)-1α. Phosphorylation of profilin-1 (Pfn-1) at Tyr 129 in ECs induces binding to the tumour suppressor protein von Hippel-Lindau (VHL), and prevents VHL-mediated degradation of prolyl-hydroxylated HIF-1α, culminating in HIF-1α accumulation even in normoxia. Elevated HIF-1α induces expression of multiple angiogenic factors, leading to vascular abnormality and tumour progression. In a genetic model of GBM, mice with an EC-specific defect in Pfn-1 phosphorylation exhibit reduced tumour angiogenesis, normalized vasculature and improved survival. Moreover, EC-specific Pfn-1 phosphorylation is associated with tumour aggressiveness in human glioma. These findings suggest that targeting Pfn-1 phosphorylation may offer a selective strategy for therapeutic intervention of malignant solid tumours.

MeSH terms

  • Animals
  • Autocrine Communication*
  • Brain Neoplasms / blood supply
  • Brain Neoplasms / genetics
  • Brain Neoplasms / metabolism*
  • Brain Neoplasms / pathology
  • Capillary Permeability
  • Cell Line, Tumor
  • Cell Proliferation
  • Disease Models, Animal
  • Disease Progression
  • Endothelial Cells / metabolism*
  • Endothelial Cells / pathology
  • Female
  • Glioblastoma / blood supply
  • Glioblastoma / genetics
  • Glioblastoma / metabolism*
  • Glioblastoma / pathology
  • Humans
  • Hypoxia-Inducible Factor 1, alpha Subunit / genetics
  • Hypoxia-Inducible Factor 1, alpha Subunit / metabolism*
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Mutation
  • Neoplasm Grading
  • Neovascularization, Pathologic
  • Phosphorylation
  • Profilins / genetics
  • Profilins / metabolism*
  • RNA Interference
  • Time Factors
  • Transfection
  • Tumor Burden
  • Tumor Microenvironment
  • Tyrosine
  • Up-Regulation
  • Von Hippel-Lindau Tumor Suppressor Protein / metabolism

Substances

  • HIF1A protein, human
  • Hif1a protein, mouse
  • Hypoxia-Inducible Factor 1, alpha Subunit
  • PFN1 protein, human
  • Pfn1 protein, mouse
  • Profilins
  • Tyrosine
  • Von Hippel-Lindau Tumor Suppressor Protein
  • VHL protein, human
  • VHL protein, mouse