Protein degradation and defense against neurodegenerative disease 1

Published on October 7, 2014   39 min

Other Talks in the Series: Protein Homeostasis

0:00
My name is Alfred Goldberg. I'm a professor of Cell Biology at Harvard Medical School. And today I'd like to talk about a fundamental cellular process, in which all cells use to keep alive but is particularly important in our defenses against neurodegenerative disease.
0:22
Neurodegenerative diseases include a number of the major challenges that our society faces. Parkinson's disease, Huntington's disease are the most common neurodegenerative diseases-- amyotrophic lateral sclerosis, different forms of dementia. The common feature of all these diseases is that they're associated with the accumulation in neurons of a variety of aggregated proteins. As shown in the next slide, each of these diseases, under the microscope, is associated with the appearance in neurons of inclusions of abnormal proteins that appear with age in patients and eventually trigger cell death. The actual proteins that accumulate in these aggregates vary between the diseases-- Tau in Alzheimer's, alpha-synuclein in Parkinson's disease and in Lewy body dementia, superoxide dismutase in ALS. And a variety of proteins with extended glutamine reagents are found in Huntington's and related diseases. What's of major interest is that these inclusions are all associated with a small protein ubiquitin and portions of the proteasome. These are the hallmarks of the degradative process that I would like to discuss today. In fact, they're an indication that the cells are trying, put in the patient's, clearly failing to eliminate these misfolded proteins. It's now been known for 40 years that all cells, from bacteria to mammalian cells, rapidly degrade misfolded or damaged proteins. The next slide is a bit of protein sociology. It lists the kinds of proteins that have been shown in various cells to be selectively degraded.
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Protein degradation and defense against neurodegenerative disease 1

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