Citation |
Treatment |
Model |
Dose |
Start |
Outcomes |
Survival |
Weight |
Behavior |
Neuropathology |
Diabetes |
Rotarod |
Other |
Atrophy |
Aggregates |
Other |
Wang 2005 |
siRNA–HDExon1 |
R6/2 |
5 μl transfection complex solution |
birth |
+ |
+ |
+ |
other: foot clasping |
+ |
other: open field |
+ |
|
|
|
|
|
Van Raamsdonk 2005 |
ethyl–EPA |
YAC128 |
1% |
6.5 months |
|
|
+ |
|
brain weight |
~ |
neuronal cross-sectional area |
~ |
striatal cell numbers |
~ |
striatal volume |
~ |
|
|
other: darpp-32 expression |
~ |
|
|
Nguyen 2005 |
clioquinol |
R6/2 |
30 mg/kg/d |
3 w |
+ |
+ |
+ |
|
|
+ |
|
+ |
Hunt & Morton 2005 |
gilbenclamide |
R6/2 |
5 mg/kg |
5.5 w |
~ |
~ |
~ |
|
|
|
|
+ |
rosiglitazone |
3 mg/kg |
~ |
gilbenclamide & rosiglitazone |
5 mg/kg & 3 mg/kg |
+ |
daily repeated exercise (swim tank) |
6 days/week |
5 w |
– |
|
|
– |
Hockly 2005 |
riluzole |
R6/2 females |
0.2 g/l |
5 w |
|
~ |
~ |
|
|
|
|
|
0.4 g/l |
– |
other: altered rearing |
~ |
other: grip strength |
~ |
other: progressive hypoactivity |
~ |
|
|
|
PGL–135 |
R6/2 |
0.2 g/l – 0.6 g/l |
|
N/A |
|
|
|
Popovic 2005 |
glial cell line–derived neurotrophic factor (GDNF) |
R6/2 |
intrastriatal injection |
4-5 w |
|
~ |
~ |
clasping |
~ |
groomnig |
~ |
other: open field |
~ |
|
|
|
cell proliferation: dentate gyrus |
~ |
cell proliferation: striatum |
~ |
cell proliferation: subventricular zone |
~ |
|
|
Harper 2005 |
RNA interference (shHD2.1) |
N171-82Q |
striatal and cerebellar injection |
4 w |
|
~ |
+ |
pawprint: stride length |
+ |
|
|
cerebellar granule cells |
+ |
striatum |
+ |
|
|
|
Chou 2005 |
CGS21680 (CGS) |
R6/2 |
0.5 μg/g IP |
7 w |
~ |
~ |
~ |
|
|
|
|
|
2.5 μg/g IP |
– |
+ |
brain weight |
+ |
ventricle |
+ |
|
5.0 μg/g IP |
|
+ |
Morton 2005 |
& |
tacrine |
R6/2 females |
2.7 or 3.2 mg/kg/d IP |
5 w |
+ |
+ |
~ |
t maze |
+ |
water maze: hidden distance |
+ |
water maze: visible 1: thigmotaxis |
+ |
water maze: visible 2: swim speed |
– |
water maze: visible 2: thigmotaxis |
+ |
|
|
~ |
|
+ |
moclobemide |
10 mg/kg/d IP |
creatine |
1% w/v oral (in diet) |
& |
tacrine |
2.7 or 3.2 mg/kg/d IP |
|
|
|
|
|
|
moclobemide |
10 mg/kg/d IP |
& |
tacrine |
2.7 or 3.2 mg/kg/d IP |
creatine |
1% w/v oral (in diet) |
& |
moclobemide |
10 mg/kg/d IP |
creatine |
1% w/v oral (in diet) |
tacrine |
2.7 or 3.2 mg/kg/d IP |
+ |
~ |
|
moclobemide |
10 mg/kg/d IP |
~ |
creatine |
1% w/v oral (in diet) |
Ferrante 2004 |
mithramycin |
R6/2 |
150 μg/kg IP |
3 w |
+ |
~ |
+ |
|
|
~ |
3-np striatal damage |
+ |
neuronal cell size |
+ |
|
|
Diguet 2004 |
minocycline |
3-NP |
45 mg/kg IP, twice daily |
20 w |
– |
|
– |
beam walking |
– |
motor disorder assessment |
– |
open field |
– |
pole test |
– |
|
|
|
|
|
Schiefer 2004 |
2–methyl–6–(phenylethynyl)–pyridine (MPEP) |
R6/2 |
100 mg/kg/d |
3.5 w |
+ |
~ |
+ |
|
|
~ |
|
~ |
LY379268 |
1.2 mg/kg/d |
~ |
|
~ |
Gardian 2004 |
phenylbutyrate |
N171-82Q |
100 mg/kg/d IP, 6 days/week (injection volume = 3.33 ml/kg) |
75 d |
+ |
~ |
~ |
|
|
|
cell loss: striatal neuron |
+ |
|
|
Schilling 2004 |
remacemide |
N171-82Q |
14-17.5 mg/kg |
8 w |
– |
|
~ |
|
|
|
|
|
coenzyme Q10 (coQ) |
400-500 mg/kg |
– |
+ |
celecoxib |
1500 ppm |
– |
~ |
chlorpromazine |
N171-82Q females |
1 mg/kg/day |
90 d |
~ |
|
~ |
environmental enrichment (EE) |
exercise, wheels, tubes, social interaction |
8 w |
~ |
+ |
+ |
|
Tanaka 2004 |
trehalose |
R6/2 |
0.2% oral |
3 w |
|
+ |
|
|
|
|
|
|
2% oral |
+ |
+ |
|
|
|
cell loss: cortex |
~ |
cell loss: striatum |
~ |
|
~ |
5% oral |
|
|
|
|
|
|
|
Duan 2004 |
paroxetine |
N171-82Q |
5 mg/kg/day subcutaneous |
8 w |
+ |
+ |
+ |
|
|
|
|
+ |
13 w |
+ |
|
|
|
|
Ravikumar 2004 |
rapamycin ester (CCI–779) |
N171-82Q |
20 mg/kg IP |
4 w |
~ |
– |
+ |
other: grip strength |
+ |
tremors |
+ |
wire maneuver |
+ |
|
|
|
|
|
Zucker 2004 |
gabapentin (GBP) |
R6/2 |
100 mg/kg/d |
6 w |
~ |
|
|
|
~ |
~ |
|
|
gabapentin–lactam (GBP–L) |
100 mg/kg/d |
|
hippocampus |
+ |
neocortex |
+ |
striatum |
+ |
|
Spires 2004 |
environmental enrichment (EE) |
R6/1 |
variety of non-toxic objects were placed in the home cage and changed every 2 days |
4 w |
|
+ |
+ |
|
|
|
|
|
Dedeoglu 2003 |
creatine |
R6/2 |
2% oral |
6 w |
+ |
+ |
+ |
|
brain weight |
+ |
striatum |
+ |
|
|
cell loss: striatal neuron |
+ |
|
|
8 w |
~ |
|
|
|
10 w |
~ |
~ |
|
Klivenyi 2003 |
BN82451 |
R6/2 |
30mg/kg/d |
30 d |
+ |
~ |
+ |
|
|
|
cell loss: striatal neuron |
+ |
|
|
Hockly 2003 |
suberoylanilide hydroxamic acid (SAHA) |
R6/2 |
100 mg/kg |
30 d |
|
– |
+ |
|
~ |
~ |
|
|
Ferrante 2003 |
sodium butyrate |
R6/2 females |
100 mg/kg/d |
3 w |
~ |
~ |
|
|
|
|
|
|
200 mg/kg/d |
+ |
400 mg/kg/d |
+ |
600 mg/kg/d |
1200 mg/kg/d |
|
|
3-np striatal damage |
+ |
cell loss: striatal neuron |
+ |
|
5000 mg/kg/d |
– |
|
|
|
|
|
10000 mg/kg/d |
Smith 2003 |
minocycline |
R6/2 |
1 mg/ml |
4 w |
|
~ |
~ |
|
|
|
|
|
5 mg/ml |
~ |
+ |
doxycycline |
2 mg/ml |
|
|
6 mg/ml |
~ |
+ |
Miller 2003 |
DNA vaccine |
R6/2 females |
100 μg plasmid, 2 doses 2 w apart |
5 w |
~ |
|
|
|
|
|
|
+ |
Wood 2003 |
lithium |
R6/2 |
16 mg/kg/d |
5.3 w |
~ |
– |
~ |
|
|
|
|
|
10.5 w |
+ |
+ |
S�nchez 2003 |
congo red |
R6/2 |
not stated |
9 w |
+ |
|
|
|
|
|
|
+ |
1 mg/30 g/2d IP |
|
+ |
+ |
|
|
|
168 mg/28d ICV pump |
|
Duan 2003 |
dietary restriction (DR) |
N171-82Q |
fast every other day |
8 w |
+ |
+ |
+ |
|
|
|
|
+ |
Ferrante 2002 |
coenzyme Q10 (coQ) |
R6/2 |
400-500 mg/kg |
3 w |
+ |
+ |
+ |
|
brain |
+ |
striatum neuron |
+ |
|
|
|
|
remacemide |
14-17.5 mg/kg |
& |
coenzyme Q10 (coQ) |
400-500 mg/kg |
+ |
brain |
+ |
striatum neuron |
+ |
ventricle |
+ |
|
|
remacemide |
14-17.5 mg/kg |
& |
coenzyme Q10 (coQ) |
N171-82Q |
400-500 mg/kg |
+ |
|
|
|
remacemide |
14-17.5 mg/kg |
Keene 2002 |
tauroursodeoxycholic acid (TUDCA) |
R6/2 |
500mg/kg/3d SC |
6 w |
|
|
+ |
|
|
|
|
|
Dedeoglu 2002 |
cystamine |
R6/2 |
112 mg/kg IP |
3 w |
+ |
+ |
+ |
|
brain |
+ |
striatum neuron |
+ |
|
|
|
|
225 mg/kg IP |
|
|
225 mg/kg oral |
prenatal |
Karpuj 2002 |
cystamine |
R6/2 |
1 μmol/d IP |
7 w |
+ |
+ |
|
|
|
cortex |
~ |
hippocampus |
~ |
striatum |
~ |
|
|
~ |
Clifford 2002 |
essential fatty acids (EFA) |
R6/1 |
254 mg/2d |
prenatal |
+ |
~ |
|
|
|
|
|
|
Schiefer 2002 |
riluzole |
R6/2 |
10 mg/kg/d |
3 w |
+ |
+ |
~ |
hyperactivity phase |
+ |
open field |
+ |
|
|
cerebral cortex |
~ |
striatum |
~ |
|
|
~ |
Hockly 2002 |
environmental enrichment (EE) |
R6/2 |
cardboard tube in cage |
4 w |
|
~ |
+ |
|
brain |
~ |
peristriatum |
~ |
striatum |
~ |
|
anterior cingulate cortex |
~ |
striatum |
~ |
|
|
|
bigger cage, more mice per cage, more bedding materials, toys |
+ |
brain |
~ |
peristriatum |
+ |
striatum |
~ |
|
Schilling 2001 |
& |
coenzyme Q10 (coQ) |
N171-82Q |
400-500 mg/kg |
4-8 w |
~ |
+ |
+ |
|
|
cerebellar granule cells |
~ |
|
|
|
remacemide |
14-17.5 mg/kg |
Andreassen 2001 |
creatine |
N171-82Q |
100 mg/d |
4 w |
+ |
+ |
+ |
|
brain |
+ |
striatum |
+ |
striatum neuron |
+ |
|
|
|
+ |
Andreassen 2001 |
lipoic acid |
R6/2 |
100 mg/kg/d |
~ |
|
|
|
|
N171-82Q |
+ |
2–sulpho–tert–phenyibutyinitrone (S–PBN) |
R6/2 |
120mg/kg/d |
~ |
~ |
Andreassen 2001 |
dichloroacetate (DCA) |
100 mg/kg/d |
+ |
+ |
+ |
|
~ |
+ |
N171-82Q |
|
|
|
Ende 2001 |
human umbilical cord blood (HUBC) |
R6/2 |
71-74 x 10^6 |
|
+ |
+ |
|
|
|
|
|
|
100-105 x 10^6 |
+ |
van Dellen 2001 |
cortical transplantation |
R6/1 |
|
newborn |
|
|
|
clasping |
+ |
horizontal bar |
~ |
|
|
|
|
|
Ferrante 2000 |
creatine |
R6/2 |
50 mg/d |
3 w |
+ |
+ |
+ |
|
|
|
|
|
100 mg/d |
+ |
+ |
+ |
striatum |
+ |
striatum neuron |
+ |
|
|
+ |
150 mg/d |
+ |
+ |
+ |
|
|
|
Chen 2000 |
minocycline |
R6/2 |
5 mg/kg IP |
6 w |
+ |
~ |
+ |
|
|
|
|
~ |
zVAD–fmk |
100 mg /20 g/28d ICV pump |
7 w |
|
|
|
|
YVAD–cmk |
50 mg /20 g/28d ICV pump |
~ |
DEVD–fmk |
& |
YVAD–cmk |
+ |
+ |
DEVD–fmk |
Carter 2000 |
environmental enrichment (EE) |
R6/2 |
enhanced diet |
4 w |
+ |
+ |
|
clasping |
~ |
ethologic |
+ |
open field |
~ |
|
|
|
|
|
enhanced diet + mixed housing with WT mice |
+ |
|
enhanced diet + early weaning +behavioral testing |
2.5 w |
~ |
enhanced diet + allowed to breed |
4 w |
van Dellen 2000 |
environmental enrichment (EE) |
R6/1 |
cardboard, paper, plastic objects |
4 w |
|
~ |
|
clasping |
+ |
horizontal bar |
+ |
|
peristriatum |
+ |
striatum |
~ |
|
|
|
|
Ona 1999 |
zVAD–fmk |
R6/2 |
100 mg /20 g/28d ICV pump |
7 w |
+ |
|
+ |
|
|
|
|
|
Dunnett 1998 |
striatal transplantation |
R6/2 |
|
10 w |
|
~ |
|
|
|
|
|
|