Line # |
Onset |
Pronounced |
Experiment |
Mouse Model |
Phenotype Classification |
Sub phenotype Classification |
Phenotype location |
Phenotype Characteristic / Test |
Notes |
1 |
4.5 wks |
10-13 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/hippocampus DG |
NII |
DG, dentate gyrus; NII neuronal intranuclear inclusion; onset not well documented; immunogold anti-ubiquitin ab |
2 |
11 wks |
n/d |
5: 16127709 |
R6/2 |
behavior |
motor |
organism |
grip strength, forelimb |
|
3 |
3 wks |
5-7 wks |
17: 15505789 |
R6/2 |
E-phys changes |
receptor/neurotransmitter responses |
brain/striatum/medium-sized spiny neurons |
Increased frequency of spontaneous GABAergic synaptic currents |
|
4 |
n/d |
11-15 wks |
18: 15625098 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
reduced membrane capacitance |
|
5 |
8-9 wks |
9-13.5 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
footprint test |
Gait changes seen at 8.5 wks were similar to those seen at 13.5 wks. |
6 |
7 wks |
10-11 wks |
23: 10575040 |
R6/2 |
behavior |
cognitive |
organism |
two-choice swim tank, acquisition (visual learning) |
|
7 |
8.6 wks |
12.9 wks |
5: 16127709 |
R6/2 |
Pathological/morphological |
atrophy |
brain |
brain volume decrease, ventricular |
|
8 |
8 wks |
13-14 wks |
24: 16111888 |
R6/2 |
behavior |
motor |
organism |
grip strength |
|
9 |
8.6 wks |
12.9 wks |
5: 16127709 |
R6/2 |
Pathological/morphological |
atrophy |
brain |
brain volume decrease |
|
10 |
12.9 wks |
n/d |
5: 16127709 |
R6/2 |
Pathological/morphological |
neurodegeneration |
brain/striatum |
decrease in striatal neuronal area/number |
|
11 |
6 wks |
12 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
atrophy |
heart |
atrophy |
|
12 |
10.7-12.1 wks |
n/d |
16: 11746372 |
R6/2 |
E-phys changes |
calcium channels |
brain/striatum/medium-sized spiny neurons |
reduced calcium current amplitude |
neurons from pre-symptomatic mice (5-6.5 wks) had slightly higher peak calcium currents |
13 |
n/d |
10 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/thalamus |
ENNI |
ENNI, extranuclear inclusion; immunogold anti-ubiquitin ab |
14 |
8-12 wks |
n/d |
1: 15579164 |
R6/2 |
E-phys changes |
Non-CNS changes |
neuromuscular junction |
increased mconotoxin resistance |
|
15 |
6 wks |
8 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
aggregates |
kidney |
nuclear inclusions |
|
16 |
4 wks |
6-12 wks |
6: 10369868 |
R6/2 |
Pathological/morphological |
aggregates |
brain/cortex |
NII |
NII, neuronal intranuclear inclusion; EM48 ab |
17 |
6 wks |
n/d |
5: 16127709 |
R6/2 |
behavior |
motor |
organism |
hindlimb clasping (dyskinesia) |
|
18 |
8.6 wks |
n/d |
21: 10533044 |
R6/2 |
E-phys changes |
receptor/neurotransmitter responses |
brain/striatum/medium-sized spiny neurons |
cell swelling in response to NMDA |
also observed depolarized RMP, but did not discuss age of onset |
19 |
n/d |
7-13 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/hippocampus CA3 |
ENNI |
ENNI, extranuclear inclusion; immunogold anti-ubiquitin ab |
20 |
4 wks |
6-12 wks |
6: 10369868 |
R6/2 |
Pathological/morphological |
aggregates |
brain/striatum |
NII |
NII, neuronal intranuclear inclusion; EM48 ab |
21 |
7 wks |
8.5-12.5 wks |
8: 10078572 |
R6/2 |
Pathological/morphological |
metabolic |
organism |
diabetes |
|
22 |
5-7 wks |
11-15 wks |
19: 12574425 |
R6/2 |
E-phys changes |
action potential properties |
brain/striatum/medium-sized spiny neurons |
reduction in the frequency of spontaneous EPSC’s |
EPSP, excitatory postsynaptic potential |
23 |
n/d |
6 wks |
22: 10829080 |
R6/2 |
E-phys changes |
receptor/neurotransmitter responses |
brain/striatum/medium-sized spiny neurons |
reduced dopaminergic modulation of GABAA current |
Lack of dopaminergic channel modulation was due to defects in the D1/cAMP/PKA/DARPP-32 signaling cascade. |
24 |
8 wks |
12-13 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
excessive grooming/ hyperactivity |
|
25 |
5-7 wks |
9-14 wks |
17: 15505789 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
increased input resistance |
The change at 5-7 wks was observed, but not shown to be significant |
26 |
n/d |
7- 13 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/hippocampus CA1 |
ENNI |
ENNI, extranuclear inclusion; immunogold anti-ubiquitin ab |
27 |
39 wks |
52 wks |
26: 12812983 |
YAC 128 |
Pathological/morphological |
atrophy |
brain |
brain weight loss |
5% decrease in brain wt. at 9 mo, progressing to 10% at 12 mo. |
28 |
n/d |
12-13 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
abrupt shuddering moments |
|
29 |
8 wks |
12 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
atrophy |
liver |
atrophy |
|
30 |
4.3 wks |
12.9 wks |
5: 16127709 |
R6/2 |
Pathological/morphological |
atrophy |
brain |
brain weight loss |
|
31 |
8 wks |
n/d |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
decreased anxiety (light/dark box) |
Decreased anxiety was observed for both male and female mice, and may be a function of reduced activity at 8 wks. |
32 |
5-6 wks |
10+ wks |
20: 10864968 |
R6/2 |
E-phys changes |
synaptic plasticity |
brain/hippocampus |
CA1 / reduced LTP |
LTP, long-term potentiation |
33 |
6 wks |
8 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
aggregates |
pancreas |
nuclear inclusions |
|
34 |
11-15 wks |
n/d |
18: 15625098 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
decreased membrane time constants |
time constants were slightly shorter, but not significantly shorter than wt |
35 |
8 wks |
n/d |
23: 10575040 |
R6/2 |
behavior |
cognitive |
organism |
visual cliff avoidance (visual learning) |
|
36 |
11.6-14.4 wks |
n/d |
13: 11731527 |
R6/2 |
E-phys changes |
action potential properties |
brain/striatum/medium-sized spiny neurons |
decreased AP decay slopes |
|
37 |
n/d |
11-15 wks |
18: 15625098 |
R6/2 |
E-phys changes |
potassium channels |
brain/striatum/medium-sized spiny neurons |
reduced inward potassium current density |
|
38 |
6 wks |
8 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
aggregates |
adrenal glands |
nuclear inclusions |
|
39 |
11.6-14.4 wks |
n/d |
13: 11731527 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
depolarized RMPs |
RMP, resting membrane potentials |
40 |
n/d |
39 wks |
26: 12812983 |
YAC 128 |
Pathological/morphological |
atrophy |
brain/striatum |
striatal volume |
A 15% decrease in striatal volume was observed at 9 mo. No decrease detected at 6 mo. |
41 |
4-9 wks |
14 wks |
24: 16111888 |
R6/2 |
behavior |
motor |
organism |
decreased exploratory activity |
Activity, mobility, and rearing were measured using the AM1053 activity cage system |
42 |
10 wks |
13 wks |
2: 8898202 |
R6/2 |
Pathological/morphological |
general |
organism |
survival |
A single mouse, with symptom onset at 4.5 wks died early at 6.5 wks |
43 |
12.9 wks |
n/d |
5: 16127709 |
R6/2 |
Pathological/morphological |
atrophy |
brain |
brain volume decrease, striatal |
|
44 |
9-11 wks |
n/d |
2: 8898202 |
R6/2 |
behavior |
motor |
organism |
hindlimb clasping (dyskinesia) |
|
45 |
5-6 wks |
10+ wks |
20: 10864968 |
R6/2 |
E-phys changes |
synaptic plasticity |
brain/hippocampus |
CA1 / increased LTD |
LTD, long-term depression |
46 |
26 wks |
52 wks |
26: 12812983 |
YAC 128 |
behavior |
motor |
organism |
decreased open field activity (dancenight) |
|
47 |
4 wks |
8 wks |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
decreased rearing activity |
|
48 |
4 wks |
n/d |
9: 9267033 |
R6/2 |
Pathological/morphological |
atrophy |
brain |
brain weight loss |
|
49 |
11-15 wks |
n/d |
5: 16127709 |
R6/2 |
behavior |
motor |
organism |
sustained flexure of forelimbs and hindlimbs |
|
50 |
10.7-12.1 wks |
n/d |
16: 11746372 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
reduced membrane capacitance |
|
51 |
13 wks |
13 wks |
26: 12812983 |
YAC 128 |
behavior |
motor |
organism |
increased movements |
|
52 |
12 wks |
15 wks |
1: 15579164 |
R6/2 |
Pathological/morphological |
aggregates |
skeletal muscle |
nuclear inclusions |
|
53 |
day 1 |
12.9 wks |
5: 16127709 |
R6/2 |
Pathological/morphological |
aggregates |
brain/cortex |
htt-positive aggregates |
|
54 |
52 wks |
n/d |
26: 12812983 |
YAC 128 |
Pathological/morphological |
atrophy |
brain/cortex |
cortex volume |
|
55 |
8 wks |
n/d |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
rotorod |
|
56 |
8.5 wks |
n/d |
23: 10575040 |
R6/2 |
behavior |
cognitive |
organism |
two-choice swim tank, retention (visual learning) |
|
57 |
4.5 -5.5 wks |
8.5- 9.5 wks |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
decreased running wheel activity (night) |
|
58 |
5-6 wks |
13-14 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
rotorod |
|
59 |
3-4 wks |
5-7 wks |
19: 12574425 |
R6/2 |
E-phys changes |
action potential properties |
brain/striatum/medium-sized spiny neurons |
large-amplitude events |
|
60 |
4 wks |
12 wks |
6: 10369868 |
R6/2 |
Pathological/morphological |
aggregates |
brain/cortex |
ENNI |
ENNI, extranuclear inclusion; EM48 ab |
61 |
12-16 wks |
n/d |
1: 15579164 |
R6/2 |
E-phys changes |
Non-CNS changes |
neuromuscular junction |
increased EPP failure |
EPP, end-plate potential |
62 |
7 wks |
n/d |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
grip strength |
|
63 |
12 wks |
15 wks |
1: 15579164 |
R6/2 |
Pathological/morphological |
atrophy |
skeletal muscle |
atrophy |
|
64 |
5-7 wks |
11 wks |
3: 16087879 |
R6/2 |
Pathological/morphological |
atrophy |
organism |
weight loss |
|
65 |
n/d |
7 wks |
20: 10864968 |
R6/2 |
behavior |
cognitive |
organism |
Morris Water Maze (spatial learning) |
|
66 |
n/d |
13 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/striatum |
ENNI |
ENNI, extranuclear inclusion, most pronounced in large interneurons |
67 |
10.7-12.1 wks |
n/d |
14: 11746372 |
R6/2 |
Pathological/morphological |
neurodegeneration |
brain/striatum |
medium-sized spiny neurons/smaller somata |
|
68 |
8-9 wks |
13-14 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
beam walking |
|
69 |
8.6 wks |
13.9 wks |
5: 16127709 |
R6/2 |
Pathological/morphological |
general |
organism |
survival |
Less than 2% of the mice died prematurely through cfonstant seizure activity as early as 8.6 wks. |
70 |
n/d |
10 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/hippocampus DG |
ENNI |
ENNI, extranuclear inclusion; also seen in lacunosum moleculare; immunogold anti ubiquitin ab |
71 |
3.5 wks |
6-9 wks |
11: 12115678 |
R6/2 |
Pathological/morphological |
aggregates |
brain/cortex |
NII |
NII, neuronal intranuclear inclusion; anti-ubiquitin ab, four mouse monoclonal abs, and polyclonal EM48 were used. |
72 |
5.3-6.0 wks |
11.6-14.4 wks |
13: 11731527 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
increased input resistance |
|
73 |
10 wks |
13 wks |
7: 10191337 |
R6/2 |
Pathological/morphological |
atrophy |
organism |
weight loss |
|
74 |
n/d |
7- 10 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/thalamus |
NII |
NII, neuronal intranuclear inclusion; onset not well documented, immunogold anti-ubiquitin ab |
75 |
11.6-14.4 wks |
n/d |
13: 11731527 |
R6/2 |
E-phys changes |
action potential properties |
brain/striatum |
increased stimulation required to induce EPSPs |
EPSP, excitatory postsynaptic potential |
76 |
5-6 wks |
10-13 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
swimming tank (straight line) |
|
77 |
3.5 wks |
n/d |
23: 10575040 |
R6/2 |
behavior |
cognitive |
organism |
Morris Water Maze (spatial learning) |
|
78 |
11.6-14.4 wks |
n/d |
13: 11731527 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
decreased membrane time constants |
|
79 |
10.7-12.1 wks |
n/d |
16: 11746372 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
decreased membrane time constants |
|
80 |
3 wks |
6-8 wks |
4: 16034568 |
R6/2 |
Pathological/morphological |
aggregates |
pancreas |
beta cells/ubiquitin-positive inclusions |
|
81 |
4 wks |
12 wks |
5: 16127709 |
R6/2 |
behavior |
motor |
organism |
prolonged helicoptering of hindlimbs |
|
82 |
n/d |
12-13 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
epileptic seizures |
|
83 |
n/d |
6 wks |
22: 10829080 |
R6/2 |
E-phys changes |
receptor/neurotransmitter responses |
brain/striatum/medium-sized spiny neurons |
reduced dopaminergic modulation of voltage-gated calcium current |
Lack of dopaminergic channel modulation was due to defects in the D1/cAMP/PKA/DARPP-32 signaling cascade. |
84 |
6.5 wks |
8.5-11.5 wks |
23: 10575040 |
R6/2 |
behavior |
cognitive |
organism |
two-choice swim tank (reversal learning) |
|
85 |
9.9 wks |
n/d |
5: 16127709 |
R6/2 |
Pathological/morphological |
atrophy |
organism |
weight loss, female |
Age specified represents onset of significant weight loss. |
86 |
9-11 wks |
n/d |
2: 8898202 |
R6/2 |
behavior |
motor |
organism |
tremor |
|
87 |
52 wks |
78 wks |
26: 12812983 |
YAC 128 |
Pathological/morphological |
aggregates |
brain/striatum |
htt-positive aggregates |
Nuclear htt staining (antibody EM48) seen as “microaggregates” at 12 mo. Nuclear inclusions seen at 18 mo. |
88 |
5-7 wks |
n/d |
19: 12574425 |
R6/2 |
E-phys changes |
receptor/neurotransmitter responses |
brain/striatum/medium-sized spiny neurons |
Glu synaptic activity changes |
|
89 |
6 wks |
12 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
atrophy |
skeletal muscle |
atrophy |
quadriceps and soleus muscle |
90 |
10 wks |
n/d |
5: 16127709 |
R6/2 |
behavior |
motor |
organism |
increased movements |
|
91 |
n/d |
11-15 wks |
18: 15625098 |
R6/2 |
E-phys changes |
potassium channels |
brain/striatum/medium-sized spiny neurons |
reduced outward potassium current density |
|
92 |
11.6-14.4 wks |
n/d |
13: 11731527 |
R6/2 |
E-phys changes |
action potential properties |
brain/striatum/medium-sized spiny neurons |
alterations in the ability to generate trains of APs |
|
93 |
4 wks |
n/d |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
decreased climbing activity |
|
94 |
8 wks |
8 wks |
24: 16111888 |
R6/2 |
behavior |
motor |
organism |
rotorod |
Some drop off in rotorod performance seen at 4 wks |
95 |
6 wks |
12 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
aggregates |
skeletal muscle |
nuclear inclusions |
|
96 |
day 1 |
12 .9 wks |
5: 16127709 |
R6/2 |
Pathological/morphological |
aggregates |
brain/striatum |
htt-positive aggregates |
|
97 |
5.0-6.5 wks |
10.7-12.1 wks |
16: 11746372 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
increased input resistance |
|
98 |
8-12 wks |
8-12 wks |
1: 15579164 |
R6/2 |
E-phys changes |
Non-CNS changes |
neuromuscular junction |
increased EPP decay time |
EPP, end-plate potential |
99 |
9 wks |
14 wks |
4: 16034568 |
R6/2 |
Pathological/morphological |
metabolic |
organism |
diabetes |
Earliest onset of glucosuria was 9.3 wks. Glucose intolerance was detected at 9 wks. Aggressive behavioral testing (swimming) accelerated the onset of diabetes. |
100 |
6.4 wks |
12 wks |
5: 16127709 |
R6/2 |
Pathological/morphological |
neurodegeneration |
brain/striatum |
progressive neurodegeneration |
|
101 |
12 + wks |
15 wks |
1: 15579164 |
R6/2 |
Pathological/morphological |
neurodegeneration |
neuromuscular junction |
AChR defects |
onset not well documented |
102 |
6 wks |
12 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
atrophy |
kidney |
atrophy |
|
103 |
12.9 wks |
n/d |
5: 16127709 |
R6/2 |
Pathological/morphological |
neurodegeneration |
brain/striatum |
increased reactive astrogliosis |
|
104 |
5-7 wks |
11-15 wks |
18: 15625098 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
increased input resistance |
|
105 |
3 wks |
10 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/cortex |
NII |
NII, neuronal intranuclear inclusion; onset not well documented, immunogold anti-ubiquitin ab |
106 |
4 wks |
8 wks |
15: 10196370 |
R6/2 |
Pathological/morphological |
atrophy |
testes |
atrophy |
|
107 |
5-7 wks |
9-14 wks |
17: 15505789 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
reduced membrane capacitance |
The change at 5-7 wks was observed, but not shown to be significant |
108 |
n/d |
11-15 wks |
18: 15625098 |
R6/2 |
E-phys changes |
basic membrane properties |
brain/striatum/medium-sized spiny neurons |
depolarized RMPs |
|
109 |
16 wks |
20 wks |
1: 15579164 |
R6/2 |
Pathological/morphological |
general |
organism |
survival |
|
110 |
4.5-5.5 wks |
n/d |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
increased anxiety (light/dark box) |
Anxiety was only observed in male R6/2 mice at 4.5-5.5 wks. |
111 |
5-6.5 wks |
10.7-12.1 wks |
16: 11746372 |
R6/2 |
E-phys changes |
receptor/neurotransmitter responses |
brain/striatum/medium-sized spiny neurons |
increased NMDA response |
NMDA-induced peak currents and current densities were higher for in a “responsive” subpopulation of R6/2 mice neurons vs WT mouse neurons |
112 |
8 wks |
12-13 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
irregular gait |
|
113 |
n/d |
52 wks |
26: 12812983 |
YAC 128 |
Pathological/morphological |
neurodegeneration |
brain/striatum |
striatal neurons/ smaller somata |
18% decrease in neuronal area. |
114 |
52 wks |
78 wks |
26: 12812983 |
YAC 128 |
Pathological/morphological |
aggregates |
brain/striatum |
NII |
Nuclear htt staining (antibody EM48) seen as “microaggregates” at 12 mo. Nuclear inclusions seen at 18 mo. |
115 |
7 wks |
10 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/hippocampus CA3 |
NII |
NII, neuronal intranuclear inclusion; onset not well documented, immunogold anti ubiquitin ab |
116 |
5 wks |
n/d |
23: 10575040 |
R6/2 |
behavior |
cognitive |
organism |
T-maze (alternation learning) |
|
117 |
14- 14.5 wks |
16 wks |
12: 10869421 |
R6/2 |
Pathological/morphological |
neurodegeneration |
brain/cortex |
progressive neurodegeneration |
Dark neurons |
118 |
9 wks |
n/d |
5: 16127709 |
R6/2 |
behavior |
motor |
organism |
grip strength, hindlimb |
|
119 |
n/d |
3 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/hippocampus CA1 |
NII |
NII, neuronal intranuclear inclusion; onset not well documented; immunogold anti-ubiquitin ab |
120 |
n/d |
12.5 wks |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
acoustic startle/prepulse inhibition |
|
121 |
4 wks |
n/d |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
decreased open field activity (night) |
|
122 |
14 – 14.5 wks |
16 wks |
12: 10869421 |
R6/2 |
Pathological/morphological |
neurodegeneration |
brain/striatum |
progressive neurodegeneration |
Dark neurons |
123 |
5.3-6.0 wks |
11.6-14.4 wks |
13: 11731527 |
R6/2 |
E-phys changes |
action potential properties |
brain/striatum/medium-sized spiny neurons |
decreased rheobase |
rheobase, stimulus intensity to evoke action potentials |
124 |
n/d |
12 + wks |
20: 10864968 |
R6/2 |
E-phys changes |
synaptic plasticity |
brain/hippocampus |
DG / reduced LTP |
onset not well documented, DG, dentate gyrus |
125 |
8 wks |
n/d |
15: 10196370 |
R6/2 |
Pathological/morphological |
aggregates |
liver |
nuclear inclusions |
|
126 |
8.5 – 9.5 wks |
n/d |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
decreased running wheel activity (day) |
|
127 |
12 + wks |
15 wks |
1: 15579164 |
R6/2 |
Pathological/morphological |
neurodegeneration |
neuromuscular junction |
nerve sprouting defects |
onset not well documented |
128 |
5 wks |
13 wks |
5: 16127709 |
R6/2 |
behavior |
motor |
organism |
rotorod |
|
129 |
39 wks |
52 wks |
26: 12812983 |
YAC 128 |
Pathological/morphological |
neurodegeneration |
brain/striatum |
decrease in striatal neuronal number |
15% decrease at 12 mo. Decrease of total neuronal and MSN counts at 9 mo not significant, but indicate a trend. |
t
130 |
n/d |
10 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/cortex |
ENNI |
ENNI, extranuclear inclusion; immunogold anti-ubiquitin ab |
131 |
11.6-14.4 wks |
n/d |
13: 11731527 |
R6/2 |
Pathological/morphological |
neurodegeneration |
brain/striatum |
medium-sized spiny neurons/dendritic deficits |
|
132 |
14 – 14.5 wks |
16 wks |
12: 10869421 |
R6/2 |
Pathological/morphological |
neurodegeneration |
brain/cerebellum |
progressive neurodegeneration |
Dark neurons |
133 |
6 wks |
8 wks |
6: 10369868 |
R6/2 |
Pathological/morphological |
atrophy |
brain |
brain weight loss |
|
134 |
9-11 wks |
n/d |
2: 8898202 |
R6/2 |
behavior |
motor |
organism |
excessive grooming/ hyperactivity |
|
135 |
8 wks |
12 wks |
6: 10369868 |
R6/2 |
Pathological/morphological |
atrophy |
organism |
weight loss |
|
136 |
10.7-12.1 wks |
n/d |
16: 11746372 |
R6/2 |
E-phys changes |
calcium channels |
brain/striatum/medium-sized spiny neurons |
reduced calcium current density |
neurons from pre-symptomatic mice (5-6.5 wks) had slightly higher calcium current density |
137 |
n/d |
10.86 wks |
3: 16087879 |
R6/2 |
Pathological/morphological |
general |
organism |
survival |
|
138 |
3.5 wks |
6-9 wks |
11: 12115678 |
R6/2 |
Pathological/morphological |
aggregates |
brain/striatum |
NII |
NII, neuronal intranuclear inclusion; anti-ubiquitin ab, four mouse monoclonal abs, and polyclonal EM48 were used. |
139 |
n/d |
6 wks |
22: 10829080 |
R6/2 |
E-phys changes |
receptor/neurotransmitter responses |
brain/striatum/medium-sized spiny neurons |
reduced dopaminergic modulation of AMPA current |
Lack of dopaminergic channel modulation was due to defects in the D1/cAMP/PKA/DARPP-32 signaling cascade. |
140 |
8 wks |
n/d |
7: 10191337 |
R6/2 |
behavior |
motor |
organism |
hindlimb clasping (dyskinesia) |
|
141 |
8.9 wks |
n/d |
5: 16127709 |
R6/2 |
Pathological/morphological |
atrophy |
organism |
weight loss, male |
Age specified represents onset of significant weight loss. |
142 |
12 wks |
15 wks |
1: 15579164 |
R6/2 |
Pathological/morphological |
neurodegeneration |
neuromuscular junction |
neurofilament abnormalities |
|
143 |
26 wks |
39-52 wks |
26: 12812983 |
YAC 128 |
behavior |
motor |
organism |
rotorod |
Rotorod deficits highly correlated with striatal neuronal cell loss. |
144 |
4 wks |
12 wks |
6: 10369868 |
R6/2 |
Pathological/morphological |
aggregates |
brain/striatum |
ENNI |
ENNI, extranuclear inclusion; EM48 ab |
145 |
n/d |
15-16 wks |
4: 16034568 |
R6/2 |
Pathological/morphological |
general |
organism |
survival |
|
146 |
3 wks |
4.5 wks |
10: 11353291 |
R6/2 |
Pathological/morphological |
aggregates |
brain/striatum |
NII |
NII, neuronal intranuclear inclusion; immunogold anti-ubiquitin ab |
147 |
4.5 – 5.5 wks |
8.5 -9.5 wks |
25: 16137562 |
R6/2 |
behavior |
motor |
organism |
increased breaks from running wheel (dark) |
|