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Pulling apart pulmonary hypertension

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Defective TFG-β signalling through the bone morphogen receptor type II is implicated in autosomal dominant and sporadic primary pulmonary hypertension. This fatal disorder is characterized by vascular remodelling, often triggered by the use of appetite-suppressant drugs which were, until recently, used to treat obesity.

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Figure 1: A feature of primary pulmonary hypertension is the plexiform lesion (a) that arises from disruption of endothelial cells of the pulmonary arteries.
Figure 2: The TGF-β-signalling pathway.

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Scott, J. Pulling apart pulmonary hypertension. Nat Genet 26, 3–4 (2000). https://doi.org/10.1038/79148

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