Best Idea Grant
At the 1st International Conference on Hyperphagia in 2009, PWSA(USA) challenged the research community to generate research projects that
These grants were called the “Best Idea Grant”
In 2009 PWSA (USA) selected five Best Idea Grant projects to fund from numerous applications.
Transcranial direct current stimulation (tDCS)
tDCS is a procedure whereby a weak electric direct current is transmitted into the brain via external electrodes. Previous data have shown that tDCS to the prefrontal cortex of the brain can change food craving in healthy subjects and preliminary data in 5 adults with PWS have shown encouraging results in PWS. This study being conducted at the University of Kansas School of Medicine and at Harvard University is designed to understand if tDCS is safe and effective in PWS.
Leptin Resistance in Mouse Models of Hyperphagia
This study being conducted at the University of Alberta, will examine leptin sensitivity in murine models of PWS and related disorders, including mice carrying targeted inactivation of the Snord116/MBII-85, necdin, and Magel2 PWS candidate genes, the Smith Magenis gene Rai1, and BBS genes. The long-term goal is to determine whether defective LepR signaling is responsible for hyperphagia in PWS and related genetic disorders, and possibly contributes to hyperphagia in the general population.
Brain-Derived Neurotrophic Factor in PWS & MC4R Function-Altering Mutations
Brain-derived neurotrophic factor (BDNF) is a protein that is important in nervous system development and function. BDNF is well-expressed in the hypothalamus, a key region in the brain for energy homeostasis, and appears to function downstream of the leptin-melanocortin signaling pathway to control appetite. In both animals and humans, diminished BDNF function is associated with hyperphagia and obesity. This project being conducted at the National Institute of Child Health and Human Development will study BDNF in two hyperphagic disorders: Prader-Willi syndrome and MC4R function-altering mutations.
Abnormal Proteins Drive the Hyperphagia in PWS
In a study being conducted at St Louis University, the mechanism involving the signaling process of serum proteins and/or peptides that are unique in type or magnitude compared to obese non-PWS patients or normal controls will be studied using state of the art proteonomic analysis.
Probing genes for hyperphagia in rare obesity-related syndromes
Several experienced centers of excellence, will collaborate in a study of rare genetic obesity-related disorders with hyperphagia including Prader-Willi syndrome, Alström syndrome and fragile X syndrome. The scientists propose that a “Window of Opportunity” exists to not only provide potential insights into genetic, biochemical and developmental pathways by probing the genes for hyperphagia and obesity impacting on each rare disorder, but also applicable to the growing problem of obesity in the general population. They propose that the three rare disorders will individually be associated with unique structural and functional genetic patterns identifiable with the latest microarray technology and bioinformatics tools.
For a more complete description of each of these projects please visit the PWSA (USA) website at www.pwsausa.org/research/HyperphagiaResearch.htm