Dr. Guttentag, one of the researchers working with a mouse model for Hermansky-Pudlak Syndrome, has been awarded a two-year grant by the Great Rivers affiliate of the American Heart Association to help fund her research proposal titled: Mechanisms of Lung Inflammation in Hermansky-Pudlak Syndrome.
Dr. Guttentag is investigating the natural history of HPS using mouse models. She is particularly interested in giant lamellar body formation and how it may contribute to the lung inflammation frequently seen in HPS.
The American Heart Association is particularly interested in her work for two reasons. First, advanced pulmonary fibrosis often puts a strain on the heart causing various heart problems such as cardiomyopthy. Thus, learning to better treat lung ailments could also help to reduce the number of people facing heart problems. Secondly, one of Dr. Guttentag’s research models looks at a medication commonly used to treat heart failure called amiodarone. In some people, amiodarone affects lamellar body formation and thus causes pulmonary inflammation – much like that seen in HPS.
As you might have guessed, those of us that closely follow HPS research are thrilled that Dr. Guttentag now has the funding to continue her work on our behalf.
I'd also like to point out - grin - that this is yet another example of why funding research into rare disorders can be so important to everyone. Lots of people experience heart failure, especially as they age. And, many are given amiodarone. Thus, it won't just be the HPS community benefiting from this work. It will be the roughly 120,000 other people that develop pulmonary fibrosis annually, plus these affected heart patients.
Dr. Guttentag is investigating the natural history of HPS using mouse models. She is particularly interested in giant lamellar body formation and how it may contribute to the lung inflammation frequently seen in HPS.
The American Heart Association is particularly interested in her work for two reasons. First, advanced pulmonary fibrosis often puts a strain on the heart causing various heart problems such as cardiomyopthy. Thus, learning to better treat lung ailments could also help to reduce the number of people facing heart problems. Secondly, one of Dr. Guttentag’s research models looks at a medication commonly used to treat heart failure called amiodarone. In some people, amiodarone affects lamellar body formation and thus causes pulmonary inflammation – much like that seen in HPS.
As you might have guessed, those of us that closely follow HPS research are thrilled that Dr. Guttentag now has the funding to continue her work on our behalf.
I'd also like to point out - grin - that this is yet another example of why funding research into rare disorders can be so important to everyone. Lots of people experience heart failure, especially as they age. And, many are given amiodarone. Thus, it won't just be the HPS community benefiting from this work. It will be the roughly 120,000 other people that develop pulmonary fibrosis annually, plus these affected heart patients.
Comments