Author: Andrew Rice
After diving into the literature of fibrodysplasia ossificans progressiva (FOP), I have begun to understand this debilitating disease. The sources I have collected will be placed into the following broad categories.
- Clinical FOP
To craft a presentation of this body of literature that will be accessible to a wide audience, I will need to start broadly. A couple of papers I have found discuss FOP in very macroscopic, accessible ways (Stein et al. 2018; Tian, Zhu, and Lu 2018). I believe these papers to be critically important in providing a solid foundation for my non-scientist audience on which I can build. Review articles may also be used in this portion of the project to begin introducing the disease.
- Mechanistic Elucidation
The biochemical mechanism by which FOP acts is complex and, while still not fully understood, it has explored in great detail by various papers (Lees-Shepard et al. 2018; Upadhyay et al. 2017; Hatsell et al. 2015). FOP is primarily caused by a mutation in the ACVR1 gene, specifically R206H. This mutation results in an overamplification of BMP signaling at inappropriate times and this causes ossification. These studies are important because without them, I would be unable to properly discuss the previous direction of the field of FOP study, as well as the specifics regarding the pathophysiology of this disease.
- Experimental Approaches & Treatment
How we study this disease is important. Without common model systems and methods, the field wouldn’t be where it is today. Various animal models have been used to study this disease, including mouse models, zebrafish models, and drosophila models. I plan to discuss the plethora of animal and other experimental models used to explore FOP, as well as the current treatment options available to patients. This will allow my audience to appreciate the methodology used to study FOP.
- Future Work and Promising New Insight
I hope to end this paper at the forefront of discovery regarding FOP. To do so, I have gathered sources that have come out very recently that propose new methods of treatment, new systems that may be affected by this disease, etc. While potentially theoretical and abstract, this portion of my review will bring my readers ‘up to speed’ with what exactly is going on with FOP right now.
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