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Anomalies of the Aging Brain: Huntington's Disease

by Georgina Cornwall, PhD
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    Now, let’s switch gears a little bit and look at Huntington’s disease. Huntington’s disease is also a neurodegenerative disorder. This one has a genetic component that we have previously addressed. It also affects the basal ganglia. But remember the dog. I reminded you or let you know about is the thalamus, this example. So we have the dog in the thalamus and he has some stimulation, dopamine. He’s really happy about his little dopamine biscuits and is very excited. Then we had the leash running through the indirect pathway, sort of keeping him under control. Huntington’s disease has more to do with a leash that gets a little bit loose. So now we have an excited puppy who’s got his dopamine biscuits that is jumping all over the brain in somewhat of an uncontrolled manner. Huntington’s disease is also a movement disorder but it is more on the opposite end of the spectrum than Parkinson’s, so movements that are uncontrollable or inappropriate. The leash is a little loose. Signals are getting sent that perhaps shouldn’t be sent. It tends to onset between 30 and 50 years of age but can also be juvenile onset. You’ll recall it is one of those expanding trinucleotide repeats. Here, we have CAG with increasing generations or the more generations that pass, we can expand this repeat or this repeat does expand. Once we reach greater than say, 40 repeats, it tends to get expressed as Huntington’s disease, an autosomal dominant disease. We end up with this mutant huntingtin protein which has additional glutamines because CAG will end up coding for glutamine. Glutamines have pretty sticky tails on them. So, the more glutamines you get in the sequence, the more the tails stick together and the more the protein will clamp. That tends to result...

    About the Lecture

    The lecture Anomalies of the Aging Brain: Huntington's Disease by Georgina Cornwall, PhD is from the course Aging.


    Included Quiz Questions

    1. Both Huntington's disease and Parkinson's disease are due to interrupted neuronal signalling in the indirect movement pathway.
    2. Both Huntington's disease and Parkinson's disease are neurodegenerative disorders.
    3. Both Huntington's disease and Parkinson's disease pathology starts in the basal ganglia with abnormal protein aggregating in the neurons.
    4. Both Huntington's disease and Parkinson's disease are progressive and result in dementia in the end-stages of the progression of the disease.
    5. Both Huntington's disease and Parkinson's disease are movement disorders.
    1. Typical onset for Huntington's disease is in age greater than 50 years old.
    2. Huntington's disease is autosomal dominant
    3. Huntington's disease is due to abnormal trinucleotide repeat expansion on the HTT gene
    4. Huntington's disease causes coding for mutant amino acid glutamine that forms sticky aggregates of protein in neurons.
    5. Huntington's disease affects basal ganglia neurons.
    1. Difficulty initiating movement
    2. Tremor
    3. Clumbsiness
    4. Muscle spasms
    5. Restlessness

    Author of lecture Anomalies of the Aging Brain: Huntington's Disease

     Georgina Cornwall, PhD

    Georgina Cornwall, PhD


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