Table 1.
Review of Immunohistochemical Studies on Postmortem Huntington Disease Central Nervous System Using Anti-Huntingtin Antibodies
| Reference | Antibody | Location of epitope in Htt amino acid sequence |
Dx | Locations studied | Patterns of IR |
|---|---|---|---|---|---|
| (6) | Ab1 | 1 – 17 aa | HD (n=9) | Cortex | I, N |
| Striatum | I | ||||
| HD (n=9), Nl (n=5) | Globus pallidus, cerebellum | - | |||
| Nl (n=5) | Cortex, striatum | - | |||
| Ab585 | 585 – 725 aa | HD (n=9), Nl (n=5) | Cortex, striatum, globus pallidus, cerebellum | C | |
| (7) | Ab585 | 585 – 725 aa | HD (n=12) | Striatum, globus pallidus | I, C |
| HD (n=12), Nl (n=8) | Cortex | I, C, N in white matter | |||
| Basal forebrain, hippocampus, cerebellum, substantia nigra | C | ||||
| Nl (n=8) | Striatum, globus pallidus | C | |||
| (8) | AP78, AP194 | 1 – 17 aa | HD (n=20) | Neocortex, striatum, dentate nucleus, amygdala, hippocampus, red nucleus | I |
| Thalamus, substantia nigra, olivary complex of medulla, Purkinje cells | - | ||||
| Nl (n=4), DC (n=10) | Neocortex, striatum, cerebellum | - | |||
| AP81 | 650 – 663 aa | HD (n=20), Nl (n=4), DC (N=10) | Neocortex, striatum, cerebellum | C | |
| (9) | 4C8 | 414 – 503 aa | HD (n=5), Nl (n=5) | Cortex, striatum, cerebellum | C, N |
| (10) | EM48 | 1 – 256 aa with 2 glutamines and deletion of polyproline stretch | HD (n=12) | Cortex | I, C, N |
| Striatum, hypothalamus, thalamus, substantia nigra, cuneate nucleus | Infrequent I, C, N | ||||
| Globus pallidus, hippocampus, cerebellum | Rare I, C, N | ||||
| Nl (n=4) | Not specified | Faint C | |||
| DC (AD n=3) | Cortex, hippocampus | Plaques | |||
| mHD549 | 549 – 679 aa | HD (n=1) | Insular cortex, striatum | C, N | |
| (11) | Antiserum 7 | 1 – 19 aa | HD (n=7) | Cortex, striatum, hippocampus | I, C, N |
| Dentate gyrus, globus pallidus, cerebellum, substantia nigra | C | ||||
| DC (n=2) | Cortex | C | |||
| Antiserum 93 | 1929 – 2421 aa | HD (n=7), DC (n=5) | Cortex, striatum, hippocampus, globus pallidus, cerebellum, substantia nigra | - | |
| (12) | CAG53b | 1 – 118 aa with 51 glutamines | HD (n=6) | Frontal cortex | I, C, N |
| Nl (n=2), DC (AD n =5) | Frontal cortex | - | |||
| HD1 | 1 – 173 aa with 23 glutamines | HD (n=6) | Frontal cortex | C, N | |
| Nl (n=2), DC (AD n =5) | Frontal cortex | - | |||
| HP1 | 80 – 113 aa | HD (n=6) | Frontal cortex | I, C, N | |
| Nl (n=2), DC (AD n =5) | Frontal cortex | - | |||
| P2 | 1187 – 1207 aa | HD (n=6), Nl (n=2), DC (AD n=5) | Frontal cortex | - | |
| HF1 | 1981 – 2580 aa | HD (n=6) | Frontal cortex | “Faint synaptic staining” | |
| Nl (n=2), DC (AD n =5) | Frontal cortex | - | |||
| anti-Hunt3 | 2110 – 2121 aa | HD (n=6), Nl (n=2), DC (AD n =5) | Frontal cortex | C | |
| (13) | 1259, 2B4 | 1 – 82 aa | HD (n=6) | Frontal cortex | I, C |
| Nl (n=3) | Frontal cortex | C | |||
| 1H6 | 115 – 129 aa | HD (n=6), Nl (n=3) | Frontal cortex | C | |
| 214 | 214 – 229 aa | HD (n=6), Nl (n=3) | Frontal cortex | C | |
| 4C8 | 414 – 503 aa | HD (n=6), Nl (n=3) | Frontal cortex | C | |
| (14) | not named | 1 – 17 aa | HD (n=8) | Middle frontal gyrus | I, C |
| Nl (n=8) | Middle frontal gyrus | - | |||
| (15) | mAb5374 (Chemicon) | 1 – 256 aa with deletion of glutamine stretch | HD (n=7) | Motor and prefrontal association cortex | I, C, N |
| (16) | mAb5374 (Chemicon) | 1 – 256 aa with deletion of glutamine stretch | HD (81 CAG# confirmed cases) | Cortex | I |
| DC (4 cases with confirmed normal CAG#) | Cortex | - | |||
| (17) | Htt1-17 | 1 – 17 aa | HD (n=6) | Parietal cortex | N |
| Nl (n=5) | Parietal cortex | - | |||
| N-Del | Portions of N-terminal 1– 90 aa excluding most of polyglutamine domain and all of first polyproline repeat | HD (n=6) | Parietal cortex | N | |
| Nl (n=5) | Parietal cortex | - | |||
| Htt55-65 | 55 – 65 aa | HD (n=6) | Parietal cortex | N | |
| Nl (n=5) | Parietal cortex | - | |||
| Htt81-90 | 81 – 90 aa | HD (n=6) | Parietal cortex | N | |
| Nl (n=5) | Parietal cortex | - | |||
| 1H6 | 115 – 129 aa | HD (n=6), Nl (n=5) | Parietal cortex | C |
HD, Huntington disease; Nl, normal control cases; DC, diseased control cases; IR, immunoreactivity; I, intranuclear; C, cytoplasmic; N, neuropil.