The Huntington’s Disease Society of America has selected Dr. Neil Aronin, a professor of medicine, to be this year’s recipient of its prestigious Research Award in recognition of his transformative contributions to what is now known about Huntington’s disease, a rare, inherited neurodegenerative condition.
Dr. Aronin is currently the chief of endocrinology and metabolism of the Department of Medicine, and the co-director of the Neurotherapeutics Institute. He is also a board member at the Huntington’s Disease Society of America’s and the Hereditary Disease Foundation’s scientific advisory boards.
“As an enthusiastic teacher, clinician and researcher, Dr. Aronin is a pioneer in clinical science and a true champion for families affected by HD,” said Louise Vetter, the CEO of the Huntington’s Disease Society of America.
Dr. Aronin’s exemplary research into Huntington’s disease has spanned three decades, with his most recent work focusing on exploring the disease’s mechanisms of pathogenesis, such as mutant Huntington mRNA kinetics, vesicle recycling and roles of proteins that interact with the expanded Huntington protein.
At present, he is exploring gene silencing for Huntington’s disease, along with a way to use viral delivery of next-generation treatments that could be studied and modified further to address a wider range of autosomal dominant conditions.
Huntington’s disease is a debilitating neurodegenerative disease that eventually takes away patients’ ability to move independently, as well as think and feel normally. Many people diagnosed with Huntington’s disease will begin to manifest the disease in their 30s or 40s, but those whose onset begins before 20 years of age often experience more rapid disease progression. The medications currently available only help in symptom management. None have been proven to have the ability to prevent Huntington’s physical, mental, and behavioral degeneration.
In an earlier report on Huntington’s disease, researchers from Baylor College of Medicine (BCM) and Stanford University in California found that in the cytosolic space of eukaryotic cells, there is a protein referred to as TriC, which is part of an aggregate that acts as a chaperonin. Chaperonins are molecules that assist in the proper folding of other proteins, while TRiC has been shown to inhibit the aggregation of mutant huntingtin proteins. These findings are available in the open access journal eLife.