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Stanley B. Prusiner

Presented by WeiJun VanLith

Early Life

Early Life

Born on May 28th, 1942 in Des Moines, Iowa. He spends most of his childhood in Des Moines, Iowa, and Cincinnati, Ohio.

Education

Walnut Hills High School

University of Pensylvania

  • B.A. in Chemistry
  • Research project focused on hypothemia in the Department of Surgery under Sidney Wolfson

University of Pensylvania School of Medicine

  • M.D.

University of California, San Francisco

  • internship
  • Residency in Neurology (started in July 1972)

Career

National Institute of Health (NIH)

  • Studied glutaminases in E. coli in the laboratory of Earl Stadtman

Staff member of UCSF after completing his residency in 1974.

  • in the neurology department

In 1999, he became the director of the institute for Neurodegenerative Diseases research laboratory at UCSF.

  • Worked on prion disease, Alzheimer's disease and tauopathies

Research

Studied the surface fluorescence of brown adipose tissue in Syrian golden hamsters under Britton Chance during 2nd year of medical school.

  • Significant changes in fluorescence of brown fat were found during non-shivering thermogenesis.

Studied the metabloism of isolated brown adipose tissue under Olov Lindberg at Wenner-Gren Institute of Stockholm during 4th year of medical school.

Studied scrapie agent at UCSF in July 1974 after he was offered an associate professor position in the Neurology department.

  • His interest in scrapie was inspired by a patient who presented with Creutzfeldt-Jakob disease during his residency rotation.

Research and Experiments

Complications

High risk pertaining to scrapie studies due to assay problems.

Initial grant was written under glutamate metabolism.

Withdrawal of support from the Howard Hughes Medical Institute (HHMI)

Data suggested the scrapie agent contains proteins instead of nucleic acids.

Nobel Prize

Prushiner won the 1997 Nobel prize in medicine and physiology for the discovery of prion.

  • coined the term "prion" in his paper published in 1982 to describe the proteinaceous infectious particles

Initial finding was controversial within the scientific community.

In 1984, Leroy Hood sequenced a portion of amino acids in prion

  • opened doors for molecular biology
  • discovery of normal form of prion protein (PrP) and mutations in the PrP gene

Nobel Prize

Prion

05

04

03

It was revealed that the protein of prion was derived from a normal protein encoded by PrP gene.

  • mutation of PrP gene was found in Creutzfeldt-Jakob disease (CJD) and Gerstmann-StrŠussler-Scheinker disease

Prion replication is a complicated process

  • carrier of PrP gene mutation will most likely develop prion disease
  • Mutant PrP encoding gene seems to destabilize the "C" shape of PrP and render it more susceptible to folding into the "S" form
  • accumulation of "S" form can lead to degenerative diseases

Prion

02

01

Normal PrP is found in a helical shape, whereas the disease-associated form exists as a flattened sheet.

  • shape determines protein activity

conclusion & discussion

conclusion

&

discussion

Why did I pick him?

How has my perception has changed as a result of my study?

Questions?

Questions

Reference

“Alzheimer's Disease.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 19 Feb. 2022, https://www.mayoclinic.org/diseases-conditions/alzheimers-disease/symptoms-causes/syc-20350447.

Norris, Jeffrey. “UCSF's Stanley Prusiner Named to Receive the 1997 Nobel Prize in Physiology or Medicine.” UCSF's Stanley Prusiner Named to Receive the 1997 Nobel Prize in Physiology or Medicine | UC San Francisco, 6 Oct. 1997, https://www.ucsf.edu/news/1997/10/131436/ucsfs-stanley-prusiner-named-receive-1997-nobel-prize-physiology-or-medicine.

“Prusiner Wins Nobel for Prions.” Science, 6 Oct. 1997, https://www.science.org/content/article/prusiner-wins-nobel-prions.

Prusiner, Stanley B. “The Nobel Prize in Physiology or Medicine 1997.” NobelPrize.org, 1998, https://www.nobelprize.org/prizes/medicine/1997/prusiner/facts/.

“Stanley B. Prusiner, MD.” Stanley B. Prusiner, MD | Institute for Neurodegenerative Diseases, https://ind.ucsf.edu/ind/aboutus/faculty/prusiners.

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