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. 2017 May 5;72(12):1164–1166. doi: 10.1136/thoraxjnl-2017-209982

Figure 1.

Figure 1

Protein Von Hippel-Lindau (pVHL) regulates hypoxia inducible factor-α (HIFα)-dependent gene transcription. Under normoxic conditions, prolyl hydroxylase (PHD) hydroxylates HIFα in the presence of iron. pVHL recognises hydroxylated HIFα leading to ubiquitination and degradation in the proteasome. In hypoxia, HIFα is not hydroxylated and therefore not recognised for degradation, resulting in transcription of HIFα-dependent genes. When pVHL is mutated, HIFα is hydroxylated but not degraded, leading to accumulation of HIFα. As a result, HIFα and hypoxia-dependent genes are constantly transcribed in normoxia. EPO, erythropoietin; iNO synthase, inducible nitric-oxide synthase; LDH, lactate dehydroxygenase; VEGF, vascular endothelial growth factor.