Huntington Research - Translational Neuroendocrine Research Unit

The Translational Neuroendocrine Research Unit (TNU) focuses on Huntington’s disease (HD) and the spectrum of amyotrophic lateral sclerosis (ALS) and the behavioural variant of frontal temporal dementia (bvFTD).

These are fatal neurodegenerative disorders without effective disease-modifying therapies. Although research has previously focused on the well known disturbances in motor function for HD and ALS, emerging studies point to early non-motor features with altered emotional processing, metabolic dysfunction and changes in sleep.

The underlying hypothesis for the research program of TNU is that early changes in these disorders constitute of hypothalamic dysfunction and pathology. Already published studies by TNU have shown pioneering data on hypothalamic pathology in these disorders. Ongoing and future studies aim to determine:

• The cellular and molecular mechanisms of hypothalamic dysfunction in HD and ALS/FTD
• The casual relationships between expressing of disease-causing genes in the hypothalamus and development of disease manifestations for HD and ALS/FTD
• If hypothalamic targets can be used in interventions to modify disease progression for HD and ALS/FTD