Mutant Mice are Making a Difference

Last week at the 4th Systemic Sclerosis World Congress the results of a study conducted by University College London and  Actelion Pharmaceuticals were presented. The findings? A sign of hope.

It was concluded that “Macitentan responsiveness supports the validity of a murine model of pulmonary hypertension in scleroderma associated with altered GFBETA/BMPRIL signaling”.

So what exactly was the study?

The study was conducted with mice…but not just regular mice. Along with normal mice, mutant mice (TβRIIδk-fib )was used that develops pulmonary hypertension(PH) once their endothelial cells are injured.

They began with four different groups of mice.  Half of these mice were the control groups for the study.

mice-800875_1920Group 1 was given treatment and Control Group one was given a control injection two days
before the induction of PH through endothelial. The other group and control group received the same…but not until 8 days after the endothelial injury was induced. The injury to the endothelial cells was caused in both normal and mutant mice using SU5416. This Increased cell division and expansion in the mutated mice.

Treatment lasted for three weeks and then the PH development was measured by examining the cardiovascular function, blood vessel architecture and the heart’s right ventricle including gene expression.

They found that the mice with the mutation all showed inflammation and thickened smooth muscle layer around their blood vessels. Researchers found vessels were obstructed 21% of the arteries they analyzed.

PH-affected mice that were not treated before the endothelial injury had right ventricles that weighed more than they should while the mice that were treated prior to the injury were normal.

All mice that received the treatment, macitentan didn’t show occlusion of their articles and presented altered gene expression in their right ventricle.

The main take-away from this study is that this treatment could both prevent and treat tissue and lung problems that are often present with PH.

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