Inhibition of hyaluronan synthesis attenuates pulmonary hypertension associated with lung fibrosis.

ABSTRACT:

Background and purpose

Group III pulmonary hypertension (PH) is a highly lethal and widespread lung disorder that is a common complication in idiopathic pulmonary fibrosis (IPF) where it is considered to be the single most significant predictor of mortality. While increased levels of hyaluronan have been observed in IPF patients, hyaluronan-mediated vascular remodelling and the hyaluronan-mediated mechanisms promoting PH associated with IPF are not fully understood.

Experimental approach

Explanted lung tissue from patients with IPF with and without a diagnosis of PH was used to identify increased levels of hyaluronan. In addition, an experimental model of lung fibrosis and PH was used to test the capacity of 4-methylumbeliferone (4MU), a hyaluronan synthase inhibitor to atte

SUBMITTER: Collum SD 

PROVIDER: S-EPMC5595757 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature