Severe hepatopathy and neurological deterioration after start of valproate treatment in a 6-year-old child with mitochondrial tryptophanyl-tRNA synthetase deficiency

Table 1 OXPHOS activities in liver homogenate from the proband measured by spectrophotometric analysis

OXPHOS COMPLEXES	Activity (Control range)	Ratio/CS	Z- score
Complex I	12 (9–49)	0.50	−1.65
Complex II	135 (52–202)	0.98	−0.89
Complex II + III	61 (18–70)	0.82	0.16
Complex III	52 (42–123)	0.79	−2.33
Complex IV	60 (31–174)	0.82	−1.85
Citrate synthase	148 (50–122)	–	–

Legend: Activities are shown as nmol/min/mg protein. The control range (n = 30) is shown as (P5-P95). The ratios of the OXPHOS complexes over citrate synthase (Ratio/CS) after logarithmic transformation are calculated, with their respective Z-score. Z-scores lower than −1.96 are significantly different (P < 0.05) from the result in control samples and are indicative of a significantly decreased OXPHOS activity (shown in bold)

ISSN: 1750-1172