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Menkes disease and infantile epilepsy

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The purpose of the semi-acute treatment, described here, is to pulse the animals with a high dosage of metabolite over a short period of time by soaking animals in a metabolite solution followed by high-dosage chronic exposure and is associated with protein aggregation assays associated with the strains: N2 (Bristol), SD551 ( let-60(ga89) ), CB1402 ( unc-15(e1402) ), UA49 ( baIn2 [P unc-54 :: -synuclein::GFP, rol-6 ( su1006 )]), AM140 ( rmIs132 [P unc-54 ::Q35::YFP]), AM141 ( rmIs133 [P unc-54 ::Q40::YFP]), UA4 ( baIn4 [P unc-54 ::Q82::GFP, rol-6 ( su1006 )]), and CL4176 ( smg-1 ( cc546 ) I

Additionally, the H571 residue was implicated as being responsible for the deprotonation of the C159 thiol and a pH titration experiment completed with the H571A variant indicates that this thiol has a pka of 8.4 (Figure 8