The National Institutes of Health’s (NIH) National Institute of Neurological Disorders and Stroke (NINDS) awarded researchers at The Scripps Research Institute (TSRI) in Jupiter, Florida a $1.7 million grant to analyze which mechanisms contribute to Huntington’s disease.
Huntington’s disease is an inherited disorder which results from a malformation in the huntingtin gene – a few of its parts are repeated more often than usual in the patient’s genome, making it unstable and incapable of producing mHtt protein. Huntington’s generally attacks a small part of the brain that controls movement and destroys nerves with a barrage of toxicity, while leaving other parts unharmed. Little is known about the processes behind this damaging condition, and there is still no cure.
“In Huntington’s disease, even though mHtt is expressed throughout the brain and peripheral tissue, it causes neuronal loss and damage in a part of the brain known as the striatum, a process that is not well understood,” Srinivasa Subramaniam, the four-year study’s lead investigator and TSRI assistant professor, said in a news release. “If we’re going to develop new ways to prevent or delay the onset of the disease, we have to clearly define the mechanisms that contribute to the death of these neurons. This new grant will help us do that.”
Subramaniam has been a leader in the study of Huntington’s disease, and he and his team had previously established that “Rhes,” an activating protein, played a key role in keeping the toxicity of the disease within the striatum region of the brain.
The study will investigate the effect of the Rhes signaling pathway on mitochondria, the organelle that feeds cells with energy. Any breakdown in the mitochondria can trigger a multitude of different conditions. The lead investigator argues that the Rhes pathway could offer a target for treatment of Huntington’s: “Drugs that disrupt Rhes could alleviate Huntington’s pathology and motor symptoms,” he explained. “Clarifying the mechanisms of this signaling pathway will help us evaluate potential drug candidates for the prevention and treatment of this terrible disease.”
Additionally, Subramaniam’s lab was awarded two NINDS Research Supplements to Promote Diversity, adding up to more than $112,000. The grants are ascribed to those who improve the diversity in a research environment by recruiting post-doc fellows and students in addition to eligible investigators from underrepresented groups in health-related research.