Fig. 2: Enzyme activity and protein expression of LARS1 at 37 °C and 40 °C.

(a) Aminoacylation measured in fibroblasts of patients and controls cultured at 37 °C or 40 °C for 48 hours. Error bars indicate standard deviations. Enzyme activity measured at 40 °C differed significantly compared with controls (p values Table S3) in all patient samples. Comparison of 40 °C aminoacylation with the activity at 37 °C in the same sample showed significant decrease upon temperature shift in three patient cells (see asterisks). (b) LARS1 protein expression by western blotting in fibroblasts of patients and controls cultured at 37 °C or 40 °C during 48 hours.