Stanley Prusiner, a UCSF professor of neurology and director of the school's Institute for Neurodegenerative Diseases, is among 10 recipients named by President Obama last month.
Three other people and a team from Santa Clara-based Intel are receiving the National Medal of Technology.
All awardees will receive their medals from Obama at a White House ceremony this afternoon.
Prusiner is being recognized for his discovery and ongoing research on an infectious agent, which he named the prion. The prion causes "mad cow" disease and other fatal neurodegenerative diseases in animals and humans.
In 1997, Prusiner received the Nobel Prize in Physiology or Medicine for his discovery.
Prusiner and his colleagues are working on developing better methods for detecting the harmful agents and effective treatments for the diseases they cause.
He has also pushed for more federal funding for Alzheimer's disease research, saying more resources could help scientists make a breakthrough in their battle against the disease by 2020.
Prusiner is the fourth UCSF scientist to be awarded the National Medal of Science.