Abnormal activation of MAPKs pathways and inhibition of autophagy in a group of patients with Zellweger spectrum disorders and X-linked adrenoleukodystrophy

Table 2 Patients’ biochemical data at the moment of the study

Patient	Age (years)	C26:0 μmol/L (nv 0.25–0.65)	C26:0/C22:0 (nv 0.01–0.03)	C26:0-LysoPC μmol/L (nv < 0.08)
1	17	2.54	0.10	0.52
2	17	4.05	0.13	0.98
3	8	4.06	0.14	1.03
4	12	2.74	0.04	0.51
5	5	2.42	0.07	0.7
6	22	1.43	0.03	0.38
7	18	0.93	0.03	0.26
8	14	0.87	0.02	0.15

NFS Neurologic function score (NFS), a 25-point scale used to evaluate the severity of neurologic dysfunction. Presymptomatic are scored = 0. IQ intelligence quotient measured by Wechsler Intelligence Scales. MRI Magnetic resonance imaging, Loes score severity system is a 34-point based severity scale for brain MRI that takes into account the location and extent of disease and the presence of focal and/or global atrophy (Loes et al. 2004). GTE/GTO glycerol trioleate/glycerol trierucate

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