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After 3 days of culture at 37 C in 5% CO 2 , cell viability was measured using a methanethiosulfonate (MTS)-based assay (CellTiter96 Aqueous One Solution, Promega) and absorbance (490 nm) was read at 4 h after adding MTS reagent using a BioTek Synergy 2 plate reader (BioTek)

J Pediatr 115:577582 Roe CR, Hoppel CL, Stacey TE, Chalmers RA, Tracey BM, Millington DS (1983) Metabolic response to carnitine in methylmalonic aciduria

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