Figure 1. mEM48 preferentially binds mutant htt. (A) An N-terminal fragment of human htt used to generate the mouse monoclonal antibody mEM48. The gap represents the missing polyQ and polyP domains in this antigen. The black box indicates two glutamines (2Q). Amino acid numbers are indicated. (B) mEM48 immunostaining of the hippocampal sections from HD transgenic (R6/2) and wild-type (WT) mice at 12 wk of age. Note that the antibody specifically reacts with intranuclear mutant htt aggregates and smaller neuropil aggregates outside the nucleus. Bar, 5 μm. (C) 35S-labeled N-terminal htt (aa 1–208) fragments containing various glutamine repeats (23, 44, 75, and 130Q) were synthesized in vitro, resolved by SDS-PAGE, and revealed by autoradiography (24 h exposure). The same blot was then probed with mEM48 and visualized with ECL reagents (1 min exposure). Note that mEM48 preferentially reacts with N-terminal htt containing longer repeats.
Figure 1.

mEM48 preferentially binds mutant htt. (A) An N-terminal fragment of human htt used to generate the mouse monoclonal antibody mEM48. The gap represents the missing polyQ and polyP domains in this antigen. The black box indicates two glutamines (2Q). Amino acid numbers are indicated. (B) mEM48 immunostaining of the hippocampal sections from HD transgenic (R6/2) and wild-type (WT) mice at 12 wk of age. Note that the antibody specifically reacts with intranuclear mutant htt aggregates and smaller neuropil aggregates outside the nucleus. Bar, 5 μm. (C) 35S-labeled N-terminal htt (aa 1–208) fragments containing various glutamine repeats (23, 44, 75, and 130Q) were synthesized in vitro, resolved by SDS-PAGE, and revealed by autoradiography (24 h exposure). The same blot was then probed with mEM48 and visualized with ECL reagents (1 min exposure). Note that mEM48 preferentially reacts with N-terminal htt containing longer repeats.

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