BIOM30002 Lecture Notes - Lecture 36: Motor Neuron, Isotopic Labeling, Microglia

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3 Nov 2018
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Mouse age vs. time before falling off rotorod. Notice drug improves survival and delays onset of disease. Mice with mnd are paralysed and fall off rotorod. Histology images shows that a number of processes occur in spinal cord. drug attenuates them all (e. g. decreases astrogliosis, the amount of activated microglia) Remember sod1 loss of function is not why people with familial mnd due to sod1 mutations get sick. Increasing sod1 activity is not part of the therapeutic mechanism of action for our drug. Drug ele(cid:448)ated a(cid:272)ti(cid:448)it(cid:455) and le(cid:448)els of od (cid:373)uta(cid:374)t protei(cid:374). More mutant sod1 = more severe motor neuron pathology = more severe phenotype. Graph shows level of each species in spinal cord. Drug converts metal-deficient form to the holo form by direct cu delivery. This stabilises the sod in a normal healthy form even if it harbours a mutation. Isotopically label cu and use it as a marker to see where it goes.

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