Molecular pathogenesis of the von Hippel-Lindau hereditary cancer syndrome: implications for oxygen sensing.

Animals Cell Cycle Cell Cycle Proteins/metabolism Cullin Proteins Disease Models, Animal Elongin Extracellular Matrix/metabolism Genotype Humans Hypoxia Ligases/genetics,metabolism Neoplasms/physiopathology Oxygen/metabolism Phenotype Protein Processing, Post-Translational Transcription Factors/metabolism Tumor Suppressor Proteins Ubiquitin-Protein Ligases Ubiquitins/metabolism Von Hippel-Lindau Tumor Suppressor Protein von Hippel-Lindau Disease/genetics,metabolism,physiopathology

CUL2 protein, human Cell Cycle Proteins Cullin Proteins Elongin Transcription Factors Tumor Suppressor Proteins Ubiquitins Ubiquitin-Protein Ligases Von Hippel-Lindau Tumor Suppressor Protein Ligases VHL protein, human Oxygen