Peak Oxygen Uptake (Ml/Min)

Supplemental Data MS200200846

Tables

(E)T-1

BASELINE 6 MONTHS 12 MONTHS

PL 1678 ± 135 1571 ± 125 1604 ± 128

Peak Oxygen Uptake (ml/min) CR 1760 ± 122 1640 ± 108 1646 ± 109

TGR 1730 ± 91 1615 ± 82 1631 ± 83†

PL 112 ± 10 101 ± 9 99 ± 9

Peak Workload (Watt) CR 122 ± 10 110 ± 9 112 ± 10

TGR 118 ± 7 107 ± 6 107 ± 7†

PL 154 ± 7 145 ± 7 150 ± 5

Peak heart rate (bpm) CR 162 ± 5 151 ± 5 155 ± 5

TGR 159 ± 4 149 ± 4 153 ± 4†

PL 1.04 ± 0.02 1.08 ± 0.02 1.06 ± 0.02

Peak RER CR 1.09 ± 0.02 1.12 ± 0.02 1.13 ± 0.03

TGR 1.07 ± 0.02 1.11 ± 0.02 1.10 ± 0.02

1 Supplemental Data MS200200846

(E)T-2

PL 24 ± 4 21 ± 3 24 ± 3 MOVEMENT VARIABILITY CR 15 ± 1 21 ± 3 21 ± 3 (degrees) TG 22 ± 2 18 ± 2 21 ± 2 R

PL 22 ± 3 18 ± 3 20 ± 2 MOVEMENT ACCURACY CR 14 ± 1 22 ± 3 20 ± 2 (degrees) TG 17 ± 2 20 ± 2 20 ± 2

2 Supplemental Data MS200200846

(E)T-3

PL 0.97 ± 0.04 0.97 ± 0.04 0.96 ± 0.05 CREATININE (mg/dl) CR 0.98 ± 0.02 1.02 ± 0.03* 1.03 ± 0.03*†

PL 31 ± 3 34 ± 3 32 ± 2 UREA (mg/dl) CR 32 ± 2 34 ± 2 32 ± 1

PL 24 ± 5 22 ± 5 22 ± 6 ALANINE TRANSAMINASE (U/L) CR 18 ± 1 15 ± 1 13.5 ± 1

PL 96 ± 15 96 ± 24 84 ± 13 CREATINE KINASE (U/L) CR 75 ± 7 73 ± 6 82 ± 7

PL 1130 ± 137 1284 ± 206 1051 ± 176 URINE VOLUME (ml/24h) CR 1555 ± 182 1512 ± 141 1421 ± 197

PL 1.04 ± 0.09 0.98 ± 0.10 1.04 ± 0.10 CREATININE (g/24h) CR 1.08 ± 0.06 1.11 ± 0.06 1.08 ± 0.06

PL 0.07 ± 0.02 0.10 ± 0.03 0.11 ± 0.03 CREATINE (g/24h) CR 0.31 ± 0.06 3.27 ± 0.29* 3.05 ± 0.39*†

3 Verbessem 4

Legends to Tables

(E)T-1 Effect of creatine intake on cardiorespiratory fitness in Huntington’s disease patients

Values are mean ± s.e.m. A graded maximal cycle ergometer test was performed before (baseline) and after 6 and 12 months of placebo (PL, n=15) or creatine (CR, n=26) intake. Oxygen uptake rate, workload, heart rate, and respiratory exchange were determined at the point of volitional exhaustion

(peak).

*bpm = beats per minute; RER = Respiratory Exchange Ratio

†p<0.05 compared with corresponding baseline value

(E)T-2 Effect of creatine intake on the quality of bimanual coordination in Huntington’s disease patients

Values are mean ± s.e.m. in the placebo group (PL, n=15), the creatine group (CR, n=26) and the total group of subjects (TGR, n=41) and represent the variability and accuracy of relative phasing during the performance of a symmetrical bimanual coordination pattern at a cycling frequency of 1.15Hz. A higher score on movement variability indicates a less stable movement pattern. A higher score on movement accuracy reflects a larger deviation from the target score, which means a less accurate movement pattern

(E)T-3 Effect of creatine intake on plasma and urine biochemistry in Huntington’s disease patients

Values are mean ± s.e.m. and respresent plasma and urine contents in the placebo group (PL, n=15), the creatine group (CR, n=26) and the total group of subjects (TGR, n=41).

*p<0.05 compared with corresponding placebo value

†p<0.05 compared with corresponding baseline value