Amyotrophic Lateral Sclerosis [ALS] | Mechanism of Neuron Death

The video discusses amyotrophic lateral sclerosis (ALS), focusing on the biochemical mechanisms leading to neuron death. It explains how protein aggregates, known as inclusion bodies, form due to genetic mutations or environmental triggers. These aggregates can act like prions, spreading from cell to cell, causing oxidative stress and neuron death. The video also covers glutamate-mediated excitotoxicity, where mutant transport proteins lead to excessive calcium influx, resulting in neuron death. Additionally, it highlights the inflammatory response triggered by prions, which further damages neurons, leading to paralysis.

What are the two types of neurons that die in ALS?

• Upper and lower motor neurons

• Sensory and motor neurons

• Cerebellar and spinal neurons

• Interneurons and sensory neurons

What are inclusion bodies in the context of ALS?

• Abnormal protein aggregates

• Normal protein structures

• Genetic mutations

• Healthy neuron cells

Which of the following is NOT a common protein aggregate observed in ALS?

• TDP-43

• Dopamine

• SOD1

• FUS

What is the role of astrocytes in the context of ALS?

• They transmit action potentials

• They release neurotransmitters

• They degrade glutamate

• They form myelin sheaths

What happens when there is excessive calcium influx in motor neurons?

• The neuron experiences excitotoxicity and dies

• The neuron becomes resistant to damage

• The neuron repairs itself

• The neuron becomes more active

What is a prion in the context of ALS?

• A type of neuron

• A protein aggregate that can infect other cells

• A neurotransmitter

• A genetic mutation

How do prions contribute to the spread of ALS?

• By propagating from cell to cell

• By repairing damaged cells

• By reducing inflammation

• By forming new neurons

What effect do activated microglia have on neurons in ALS?

• They repair damaged neurons

• They decrease inflammation

• They release noxious chemicals causing damage

• They enhance neuron function

What is the significance of the term 'sclerosis' in ALS?

• It indicates neuron hardening due to damage

• It refers to muscle growth

• It describes a type of neurotransmitter

• It means increased neuron flexibility

What does 'amyotrophic' refer to in ALS?

• Increased muscle activity

• Lack of muscle changes

• Decreased neuron activity

• Changes in muscle structure