Table 1.
Study | ALS cases (n) |
Controls | Results |
---|---|---|---|
Tohgi et al. 1999(81) |
19 sALS | 19 HC | ↑CSF 3-NT and 3-NT/T ratio in sALS |
Tohgi et al, 1999 (82) |
18 sALS | 14 HC | ↑NO3 in sALS, ↑GSSG/GSH ratio in sALS |
Bonnefont- Rousselot et al, 2000 (83) |
167 | 62 HC | ↑TBARS in ALS, ↑ SOD1 activity in ALS, no other significant differences |
Ryberg et al, 2004 (84) |
14 | 19 HC, 17 AD | No increasing in ALS |
Sohmiya et al, 2005 (85) |
20 sALS | 20 HC | ↑ oxidized form of coQ10 in sALS and correlation with disease duration, no others significant differences. |
Yoshino et al, 2006a (74) |
20 | / | ↓ CSF 3NT levels at the end of antioxidant therapy |
Siciliano et al, 2007 (86) |
44 sALS, 4 fALS |
8 OND | ↓ FRA in ALS CSF ↑ AOPP in CSF and plasma ALS |
Murata et al, 2008 (87) |
30 sALS | 17 HC | ↑ percentage of oxidized CoQ10 in CSF sALS, correlation oxidized CoQ10 and duration of sALS, not correlation with the ALS score |
Keizman et al, 2009 (88) |
86 | 86 HC | ↓ ALS uric acid level; correlation serum uric acid levels and rate of progression ALSFRS-R |
Cova et al, 2010 (89) |
88 sALS | 50 HC | ↓ all assayed enzymes and correlation with progression rate in sALS, plasma ROS significantly lower in sALS |
Mendonça et al, 2011 (90) |
10 | 6 HC | No increased levels in ALS |
AD = Alzheimer disease, sALS = sporadic amyotrophic lateral sclerosis, fALS = familial amyotrophic lateral sclerosis, ALSFRS-R = Amyotrophic Lateral Sclerosis Functional Rating Scale- revised, AOPP = advanced oxidation protein products, CAT = catalase, CoQ10 = coenzyme Q10, CSF = cerebrospinal fluid, FRA = ferric reducing ability, GPX = glutathione peroxidase, GR = glutathione reductase, GSH = glutathione, GSSG = Glutathione disulfide, GST = glutathione transferase, G6PD = glucose-6-phosphate dehydrogenase, HC = healthy controls, HNE = 4-hydroxy-2,3-nonenal, F2-IsoPs = F2-isoprostanes, ND = neurodegenerative diseases, NND = non-neurodegenerative disease, ROS = reactive oxygen species, SOD = superoxide dismutase, T = tyrosine, TAS = serum total antioxidant status, TBARS = thiobarbituric acid-reactive substances, 3-NT = 3-Nitrotyrosine, 8-oxodG = 8-oxo-deoxyguanosine
Phase II clinical trial: 20 subjects with ALS received either 30 mg (5 subjects) or 60 mg (15 subjects) of edaravone (free radical scavenger) via intravenous drip once per day. Two weeks of administration was followed by a two-week observation period, for six times. In almost all patients in the 60 mg group, the level of CSF 3NT, was reduced at the end of the sixth cycle of administration.