Table 4.
Summary of SCA fluid biomarkers
| Biomarker | SCA cases vs. controls | Presymptomatic SCA cases vs. controls | Presymptomatic SCA cases vs. symptomatic SCA cases | Adult-onset SCA cases vs. early-onset SCA cases* |
|---|---|---|---|---|
| Poly-Q expanded ataxin-3 | ↑ in PBMC of SCA3 [80] ↑ in plasma and CSF of SCA3 [82] |
↑ in PBMC of presymptomatic SCA3 [80] ↑ in plasma and CSF of presymptomatic SCA3 [82] |
↑ in CSF of symptomatic SCA3 vs. presymptomatic SCA3 [82] |
|
| Catalase activity | ↑ in serum of SCA3 [91] | |||
| CHIP | ↑ in serum of SCA3 [83] ↑ in CSF of SCA3 [83] |
|||
| Oxidation of DCFH-DA | ↑ in serum of SCA3 [92] | ↑ in serum of presymptomatic SCA3 [92] |
↑ in serum of symptomatic SCA3 vs. presymptomatic SCA3 [92] |
|
| Eotaxin | ↑ in serum of symptomatic SCA3 vs. presymptomatic SCA3 [84] |
|||
| GFAP | ↑ in serum of SCA3 [139] | |||
| Glutathione peroxidase activity | ↓ in serum of symptomatic SCA3 [92] |
↓ in plasma of symptomatic SCA3 vs. presymptomatic SCA3 [92] |
||
| IGFBP-1 | ↑ in serum of SCA3 [94] | |||
| IGFBP-3 | ↓ in serum of SCA3 [94] | |||
| IGF-1/IGFBP-3 molar ratio | ↑ in serum of SCA3 [94] | |||
| Insulin | ↓ in serum of SCA3 [94] | |||
| miRNA | ↑ miR-34b [85] in serum of SCA3 |
Alterations of miRs in plasma of early onset SCA7 vs. adult onset SCA7 [97] |
||
| ↑miR-7014 in CSF of SCA1, SCA2, and SCA3 [96] ↑71 miRs in plasma of SCA7 [97] ↓miR-25 [85], miR-29a [85], miR-125b [85] in serum of SCA3 ↓ miR-7014 [96] in plasma of SCA3 Different expression of various exosomal miRs in plasma and CSF of SCA3 [96] |
||||
| Neurofilament light chain | ↑ in serum of SCA1 [78] and SCA3 [78–80] ↑ in CSF of SCA3 [79] |
↑ in serum of presymptomatic SCA3 [79, 80] |
||
| NSE | ↑ in serum of SCA3 [86, 87] | |||
| Phosphorylated neurofilament heavy chain | ↑ in serum of SCA3 [80] | |||
| S100B | ↑ in serum of SCA3 [86] | |||
| Superoxide dismutase activity | ↓ in serum of symptomatic SCA3 vs. presymptomatic SCA3 [92] |
|||
| Tau | ↑ in CSF of SCA2 [77] | |||
| Valine, leucine, and tyrosine | ↓ in plasma of SCA7 [95] |
CHIP, carboxyl terminus of the Hsp70-interacting protein; DCFH-DA, 2’,7’-dichlorofluorescein diacetate; GFAP, glial fibrillary acidic protein; GSH-Px, glutathione peroxidase; IGFBP, insulin-like growth factor-binding protein; IGF, insulin-like growth factor; miRNA, microRNA; NSE, neuron-specific enolase; S100B, protein S 100 B; SOD, superoxide dismutase.
*
Adult-onset ≧ 20 years old, early-onset < 20 years old