Mutant Huntingtin (mHTT) and Treatment of Huntington's Disease

by Georgina Cornwall, PhD

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    Let’s take a little closer look about what we know about the huntingtin protein or mHTT. It results in a multitude of different effects. First of all, it appears to be clearly most toxic in the brain. Anything that has a neurodegenerative effect clearly is going to impact the brain before anything else. But we also see that it’s affecting not only connections between the basal ganglia but also it starts affecting connections further out into the brain which is where we see dementia setting in in later stages in the progression. Again, this mHTT forms clusters in neurons. So we have a common theme with all of these disorders of aging. We have dysfunctional proteins being formed that tend to stick together and aggregate forming clusters that get in the way of communication between the cells. We can see also the clusters get in the way of regular transcription, so proteins, other proteins, tend to start to be degenerative proteins that are not in their normal form. We also see there are mitochondrial disorders. So along with the neuronal disorders, we have disorders with transcription of other genes and then destruction of mitochondria which result because somehow the caspase system is activated. You probably, “Well, what does caspase get involved in?” Caspase, you may recall, has to do with apoptosis. The mitochondria basically send out caspase to say like, “Whoa! Help me. Rescue me. I’m toxic.” Then the immune system will be activated to come along and destroy them. So, caspase is a signal that mitochondria use to signal that it’s time for that cell to be degraded. That would be a great explanation of why we see the neurons actually being destroyed in those connections. Let’s finally consider what we do in treatment of Huntington’s disease. The sad...

    About the Lecture

    The lecture Mutant Huntingtin (mHTT) and Treatment of Huntington's Disease by Georgina Cornwall, PhD is from the course Aging. It contains the following chapters:

    • Mutant Huntingtin (mHTT)
    • Treatment of Huntington's Disease

    Included Quiz Questions

    1. mHTT causes mitochondrial dysfunction.
    2. mHTT is toxic only in the brain.
    3. mHTT does not affect the cortex of the brain.
    4. mHTT only affects neurotransmitter communication between sub-cortical neurons.
    5. mHTT blocks regular translation of genes.
    1. Tetrabenazine
    2. Levodopa
    3. Carbidopa
    4. Tetracycline
    5. Acetylcholine
    1. There is no treatment for Huntington's disease; there is only symptomatic relief through a multidisciplinary team approach.
    2. Tetrabenazine
    3. Levodopa/Carbidopa
    4. Deep Brain Stimulation
    5. Speech and occupational therapy alone

    Author of lecture Mutant Huntingtin (mHTT) and Treatment of Huntington's Disease

     Georgina Cornwall, PhD

    Georgina Cornwall, PhD

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