NEURO410 Lecture Notes - Lecture 18: Superoxide Dismutase, Motor Neuron Disease, Glutathione Peroxidase

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Lecture 7: als (cid:862)lou gehrig"s disease(cid:863) (march 15/16) Overview: intro/definitions, etiology and diagnosis, superoxide dismutase, affected motoneurons, motoneuron degeneration: mechs, characteristics of the disease. In 1869 described a late stage motor neuron disease. (cid:374)oted that i(cid:374) the lateral portio(cid:374)s of the spi(cid:374)al (cid:272)ord is a disti(cid:374)(cid:272)t (cid:862)(cid:373)(cid:455)eli(cid:374) pallor(cid:863) represe(cid:374)ti(cid:374)g degeneration and loss of the axons from upper motor neurons: pallor = disruption/degen. Adult-onset, ndd that preferentially (differentiates it) affects upper and lower motoneurons. Progressive muscle weakness, atrophy and spasticity (depends on where motoneurons dying) No effective treatments available and diagnosis is very difficult. Now beginning to understand the disease processes underlying preferential motoneuron death. 1. 2 motor unit: a lower motoneuron and all the muscle fibers that it innervates. Low prevalence (b/c of aggressive nature of disease) What is really being targets: 1 motor nerve and all the motor fibers it innervates = 1 motor unit. Note: not all motor units are the same.

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