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- Clinical Introduction
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1. Frontotemporal dementia
- Prof. Bruce Miller
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2. Parkinson disease
- Prof. Stanley Fahn
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3. Atypical parkinsonian syndromes
- Dr. David Burn
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4. Huntington's disease
- Prof. Roger Barker
- Neuroimaging
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5. Molecular brain imaging (PET) in diseases with dementia
- Prof. Karl Herholz
- Pathology, Genetic and Molecular Aspects (1)
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6. A molecular understanding of Alzheimer's disease
- Prof. John Hardy
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7. Neuropathology of neurodegenerative disorders
- Prof. Jillian Kril
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9. Ubiquitination and Alzheimer related disorders
- Prof. John Mayer
- Pathology, Genetic and Molecular Aspects (2)
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10. The molecular biology of Huntington's disease
- Prof. David C. Rubinsztein
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11. Metals, oxidative stress and neurodegeneration
- Prof. Ashley Bush
- Latest Developments in the Field
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12. Animal models of tauopathy
- Prof. David Westaway
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13. Parkinson's disease and transplants
- Prof. Roger Barker
- Archived Lectures *These may not cover the latest advances in the field
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14. Neuropathology of neurodegenerative disorders
- Prof. Jillian Kril
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15. Motor neurone disease: molecular basis
- Prof. Kevin Talbot
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16. Alzheimer's disease (AD)
- Prof. John Hodges
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17. Frontotemporal dementia syndromes
- Prof. John Hodges
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18. Motor neurone disease: clinical aspects
- Prof. Kevin Talbot
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19. Neuro-imaging in dementia: using MRI in routine work-up
- Prof. Philip Scheltens
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20. Prion diseases
- Prof. Pierluigi Gambetti
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21. Mitochondrial disorders and neurodegeneration
- Prof. Anthony Schapira
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23. Mutations in parkinsonian syndromes
- Dr. Andrew Singleton
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25. Frontotemporal dementia
- Prof. Bruce Miller
Printable Handouts
Navigable Slide Index
- Intro slide
- Pathology of diseases
- Basic hypothesis
- A prescient suggestion
- Down's syndrome and Alzheimer's disease
- Alzheimer's disease
- Amyloid precursor protein and PS1
- APP metabolism
- APP mutations
- Sporadic Alzheimer's disease
- Linkage curve
- Conclusions on Alzheimer's disease
- Prion diseases
- First family with a prion mutation
- CJD
- Sporadic CJD
- Prion disease conclusions
- Tau pathology
- Tau protein
- Tau mutations cause diseases
- Alterations in exon splicing
- Tau haplotypes
- Tau & PSP
- Primary tauopathies
- Lewy bodies
- Mutations in alpha-synuclein
- Synuclein and sporadic Parkinson's disease
- Iowa kindred structure
- Alpha-synuclein pathologies
- Chromosomal spreads (FISH)
- Lewy bodies disease conclusions
- Overall conclusions
Topics Covered
- Protein pathology of disease
- Loci underlying autosomal dominant neurodegenerative disease
- Prion mutations causing Gerstmann Straussler syndrome and hereditary Creuzfeldt-Jakob disease
- Tau mutations cause autosomal dominant frontal temporal dementia
- Alpha-synuclein mutations cause autosomal dominant Parkinson's disease
- How genetic variability of these genes contributes to the risk of sporadic disease
Links
Series:
Categories:
Therapeutic Areas:
Talk Citation
Hardy, J. (2021, March 8). Neurodegenerative disease: expression levels of normal sequence pathogenic proteins contribute to risk of disease [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved December 6, 2024, from https://doi.org/10.69645/OFFC2583.Export Citation (RIS)
Publication History
Financial Disclosures
- Prof. John Hardy, Consultant: Eisai Speaker's Bureau: Eli Lily Grant/Research Support (Principal Investiqator): MRC/Wellcome Trust
Update Available
The speaker addresses developments since the publication of the original talk. We recommend listening to the associated update as well as the lecture.
- Full lecture Duration: 21:05 min
- Update Interview Duration: 20:43 min
Neurodegenerative disease: expression levels of normal sequence pathogenic proteins contribute to risk of disease
A selection of talks on Genetics & Epigenetics
Transcript
Please wait while the transcript is being prepared...
0:00
In this talk, I'm going to talk about Alzheimer's disease,
prion disease, diseases with tau pathology, and Parkinson's disease.
The point I'm going to make is that in all of these cases,
we, being the general research community,
have used a very rare or several dominant forms of the disease to elucidate pathogenesis,
to elucidate the etiology of a few cases of
disease and then to start to elucidate pathogenesis.
But these in general,
these cases of autosomal dominant disease are very rare.
We always wonder about what's causing the more common sporadic forms of the disease.
What I'm going to talk about in this talk is
the relationship between the common sporadic forms
of the disease and the autosomal dominant forms in terms of etiology and pathogenesis.
0:54
Now, these are the disease I'm going to talk about,
Alzheimer's disease characterized by A Beta plaques and tangles and often by
Lewy bodies where the Alzheimer talk I've done in
this series and plenty of other locations,
we know that the initiating molecule is the A Beta molecule.
Also we're going to talk about Prion disease where
autosomal dominant Prion disease is caused by prion mutations.
I'm going to talk about FTDP 17,
the tangle diseases, and I'll talk about the sporadic tangle diseases.
Peer progressive supranuclear palsy and corticobasal degeneration,
and I'll talk about Parkinson's disease and Lewy body dementia,
the disease is characterized by synuclein pathology and Lewy bodies.
Just a couple of notes while we have this slide up,
I regard PSP by definition FTDP 17 is a hereditary disease
and PSP and corticobasal degeneration are almost always sporadic diseases.
They overlap in clinical features with FTDP 17 and I in
a way regard them as the sporadic variant of these diseases.
I regard them as being the sporadic tangle diseases.
The other note to make is that Parkinson's disease and Lewy body dementia,
I regard as the same each eulogy entity.
Obviously, they're treated differently because
the Lewy bodies are in different positions.
But we have many families now where there are both diseases in the kindred,
illustrating that from a pathogenesis point of view they're probably related.
Most of what I'm going to say is covered in
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