Table 2.
Summary of average log-transformed protein expression levels ± SEM, Confidence Intervals (lower, upper), and [p-values] in the frontal cortex and striatum of highly expanded paediatric (HE-PHD) compared to juvenile onset HD (JOHD), adult onset HD (AOHD) and control (Ctrl) individuals.
| Protein | Cohort | Frontal cortex | Striatum |
|---|---|---|---|
| GLUT1 | HE-PHD | 12.5 ± 0.19 (10.1, 14.9) | 9.83 ± 1.30 (−6.67, 26.3) |
| Ctrl | 14.0 ± 0.19 (13.4, 14.7) [p = 0.009] | 12.8 ± 0.12 (11.2, 14.4) [p = 0.129] | |
| JOHD | 13.8 ± 0.27 (12.7, 15.0) [p = 0.035] | 11.0 ± 0.07 (10.1, 12.0) [p = 0.65] | |
| AOHD | 13.7 ± 0.21 (13.1, 14.3) [p = 0.030] | 11.7 ± 0.56 (4.57, 18.8) [p = 0.376] | |
| GLUT3 | HE-PHD | 13.1 ± 0.44 (7.50, 18.7) | 13.3 ± 0.20 (10.7, 15.9) |
| Ctrl | 14.3 ± 0.05 (14.2, 14.5) [p = 0.017] | 14.5 ± 0.02 (14.3, 14.7) [p = 0.022] | |
| JOHD | 13.0 ± 0.70 (10.0, 16.1) [p = 0.987] | 13.3 ± 0.21 (10.7, 16.0) [p = 0.999] | |
| AOHD | 13.7 ± 0.21 (13.2, 14.3) [p = 0.440] | 13.8 ± 0.15 (11.9, 15.7) [p = 0.309] | |
| Rab11-A | HE-PHD | 16.3 ± 0.02 (16.1, 16.5) | 16.5 ± 0.49 (10.3, 22.7) |
| Ctrl | 17.4 ± 0.08 (17.1, 17.6) [p = 0.011] | 17.6 ± 0.01 (17.6, 17.6) [p = 0.170] | |
| JOHD | 16.9 ± 0.34 (15.5, 18.4) [p = 0.175] | 17.3 ± 0.31 (13.3, 21.3) [p = 0.377] | |
| AOHD | 17.4 ± 0.09 (17.1, 17.6) [p = 0.010] | 17.3 ± 0.12 (15.8, 18.7) [p = 0.404] | |
| HK-II | HE-PHD | 16.7 ± 0.21 (14.0, 19.4) | 16.7 ± 0.59 (9,2, 24.2) |
| Ctrl | 18.0 ± 0.12 (17.8, 18.2) [p = 0.010] | 18.6 ± 0.01 (18.6, 18.7) [p = 0.045] | |
| JOHD | 17.5 ± 0.44 (15.6, 19.4) [p = 0.131] | 17.7 ± 0.22 (14.8, 20.5) [p = 0.283] | |
| AOHD | 17.9 ± 0.06 (17.7, 18.1) [p = 0.013] | 17.9 ± 0.14 (16.0, 19.7) [p = 0.193] | |
| Complex I | HE-PHD | 16.9 ± 0.62 (9.0, 24.8) | 18.1 ± 0.11 (17.4, 20.1) |
| Ctrl | 17.6 ± 0.19 (17.0, 18.3) [p = 0.155] | 18.7 ± 0.01 (18.7, 18.8) [p = 0.230] | |
| JOHD | 17.9 ± 0.21 (17.4, 18.5) [p = 0.049] | 18.4 ± 0.03 (18.0, 18.9) [p = 0.633] | |
| AOHD | 17.8 ± 0.06 (17.6, 18.0) [p = 0.064] | 18.8 ± 0.11 (17.4, 20.1) [p = 0.173] | |
| Complex II | HE-PHD | 16.7 ± 0.15 (14.8, 18.6) | 15.8 ± 0.19 (13.3, 18.2) |
| Ctrl | 17.4 ± 0.09 (17.1, 17.6) [p = 0.027] | 17.3 ± 0.01 (17.3, 17.4) [p = 0.024] | |
| JOHD | 17.2 ± 0.13 (16.6, 17.7) [p = 0.150] | 17.0 ± 0.33 (12.8, 21.2) [p = 0.048] | |
| AOHD | 17.3 ± 0.11 (17.0, 17.6) [p = 0.052] | 16.7 ± 0.19 (14.3, 19.2) [p = 0.099] | |
| Complex III | HE-PHD | 15.6 ± 0.41 (10.3, 20.8) | 17.6 ± 0.43 (12.1, 23.1) |
| Ctrl | 16.7 ± 0.05 (16.6, 16.9) [p = 0.002] | 19.1 ± 0.01 (19.1, 19.2) [p = 0.029] | |
| JOHD | 16.1 ± 0.21 (15.2, 17.0) [p = 0.061] | 18.4 ± 0.03 (17.9, 18.8) [p = 0.215] | |
| AOHD | 16.6 ± 0.01 (16.6, 16.7) [p = 0.002] | 18.3 ± 0.03 (18.0, 18.7) [p = 0.246] | |
| Complex IV | HE-PHD | 16.0 ± 0.75 (6.48, 25.6) | 16.2 ± 0.51 (9.76, 22.6) |
| Ctrl | 17.6 ± 0.11 (17.2, 17.9) [p = 0.217] | 18.9 ± 0.01 (18.9, 19.1) [p = 0.010] | |
| JOHD | 17.1 ± 0.94 (13.1, 21.2) [p = 0.501] | 17.5 ± 0.15 (15.6, 19.3) [p = 0.120] | |
| AOHD | 17.8 ± 0.13 (17.4, 18.1) [p = 0.123] | 17.8 ± 0.28 (14.3, 21.4) [p = 0.058] | |
| Complex V | HE-PHD | 15.7 ± 0.14 (13.9, 17.5) | 16.8 ± 0.31 (12.8, 20.8) |
| Ctrl | 16.2 ± 0.13 (15.8, 16.6) [p = 0.161] | 18.2 ± 0.02 (18.2, 28.5) [p = 0.012] | |
| JOHD | 16.2 ± 0.21 (15.3, 17.0) [p = 0.246] | 17.4 ± 0.02 (17.2, 17.6) [p = 0.172] | |
| AOHD | 16.2 ± 0.14 (13.9, 17.5) [p = 0.156] | 17.3 ± 0.07 (16.3, 18.2) [p = 0.277] |
Assessment of highly expanded (HE) Paediatric Huntington disease (PHD) compared with control (Ctrl), Juvenile-Onset (JOHD), and Adult-Onset HD (AOHD) individuals.