izABEllA PENA, PHD

Mentor: Myriam Heiman, PhD

Institution: Picower Institute for Learning and Memory

Massachusetts Institute of Technology, Cambridge

Project Title: Investigating mitochondrial defects in the most vulnerable neurons in Huntington’s disease

In Huntington’s disease a specific type of neuron in the brain dies preferentially over time. This cell type is called a “medium spiny neuron” (MSN). Dr. Pena seeks to understand why MSNs are more susceptible than other neurons to the damage that unfolds in Huntington’s disease. Considerable evidence from the scientific literature suggests that there are specific defects in the mitochondria of MSNs in HD, mitochondria being one of the cell’s components (organelles) responsible for generating cellular energy. To pursue this hypothesis she will study this tiny organelle in the MSNs of mouse models with and without the HD-causing gene expansion. Dr. Pena and colleagues developed a technique that enables purification of mitochondria from MSNs and another technology that separates mitochondria from all other neurons. She seeks to understand what is specifically vulnerable about the mitochondria of MSNs in Huntington’s disease.