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Optimal Storage Conditions Lyophilized powder: -20C to -80C in freezer Reconstituted solution: 2C to 8C in refrigerator Protected from direct light exposure Sealed containers to prevent contamination Upright positioning in storage Storage Duration Guidelines Unopened powder: 12-24 months frozen Reconstituted with bacteriostatic water: 4-6 weeks refrigerated Reconstituted with sterile water: 72 hours refrigerated After first puncture: Follow bacteriostatic water guidelines Discard if discoloration or particles appear Monitoring Solution Integrity Inspect reconstituted solutions before each use

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These studies showed significant alterations in ceramide levels as well as benzoate, creatine and fatty acid metabolisms and sphingomyelins pathways, with respect to ALS progression 49 , and these changes in metabolite levels correlated with patients ALS Functional Rating Scale (ALSFRS-R) scores 55

Chi, L., Ke, Y., Luo, C., Gozal, D