Inside ALS: The neurons behind the disease

amyotrophic Lateral sclerosis Shortened to Als is a neurodegenerative disease Which usually begins in adulthood and Advances rapidly? It's characterized by the deterioration and loss of both upper and lower motor Neurons as the motor Neurons stop sending signals the Muscles weaken leading to Paralysis When the muscles in the diaphragm are paralyzed this can be fatal. There is no known cure for Als For most people the cause of their lS is unknown, but some people inherit the disease from their parents By studying the genes that are altered in these patients scientists have pinpointed processes in the neurons that might be causing als It's too early to [know] which of these altered processes are a result of the disease and which could be a cause But it's clear that they affect many different aspects of the motor neuron function Let's start in the cell body here proteins that are not transported into the nucleus build up in the cytoplasm Errors in the systems that build up and break down proteins also cause other [Misfolded] proteins to accumulate The different proteins can then aggregate and can become toxic to cells in Several ways For example they can damage mitochondria the cell's power generators Mitochondrial damage can lead to oxidative stress which can trigger breaks in the dNA of the cell Als seems to affect Dna repair processes as well When Dna breaks are poorly repaired it ultimately contributes to the death of the neuron? the cells Transport Machinery can also be damaged For example [als] affected neurons often have problems Transporting rnA proteins and vesicles Both in the cytoplasm and along the neurons axon these vesicles containing [Pourtant] cell signaling molecules called neurotransmitters If these can't be moved along the axon or released the neuron can't send messages to its target cells damage to the cytoskeleton can also cause the Axon to retract [if] This happens the Axon can no longer connect to the muscle nearby and can no longer signal the muscle to contract other cell types can also be involved in als Oligodendrocytes which electrically insulate the axon and [provide] support to the motor neuron don't work in people with Als reduced uptake of Neurotransmitters by astrocytes can lead to over activation of the receptors at the synapse and death of the neuron Finally astrocytes and Microglia can produce factors that protect or [damaged] motor neurons Neuronal death occurs when excessive damaging factors are produced Further research is needed to find out which of these many processes caused the motor neuron degeneration seen in als patients Targeting new drug therapies at those processes might lead to a treatment for this incurable disease

Channel: nature video

Views: 172,711

Rating: 4.9500551 out of 5

Keywords: Nature, NRDP, ALS, Amyotrophic lateral sclerosis, motor neuron, motor neuron disease, Lou Gehrig, Lou Gehrig's disease, muscle, muscles, Stephen Hawking, Hawking, ice bucket challenge, disease, disease primers

Id: xrIjFVMliOQ

Length: 3min 27sec (207 seconds)

Published: Fri Apr 21 2017