Anti-HTT antibody epitopes and analysis by immunoblot.

<p>(A) Diagram representing antibody epitopes on human HTT protein (relative to GenBank accession CAD38447.1). A stretch of glutamine (Q) residues near the N-terminus is expanded in individuals affected by Huntington's disease. Amino-acid 1-92 encoded by exon-1 are shown. Blue, pAb147 antibody epitope on mouse HTT protein (GenBank accession NP_034544). (B) Immunoblot analysis of human large fragment recombinant HTT proteins detected by the indicated anti-huntingtin antibodies. 5 ng of both HTT (1–573) Q23 (lanes 1, 3, 5, 7, 9, 11, 13 and 15) and HTT (1–573) Q73 (lanes 2, 4, 6, 8, 10, 12, 14, and 16) purified large fragment proteins were analyzed by SDS-PAGE. M, molecular weight marker (kDa). (C) Immunoblot analysis of wild type littermate and transgenic BAC HD mouse whole brain extracts. Mouse endogenous wild type and human transgenic polyglutamine-expanded HTT proteins were detected using the indicated anti-huntingtin antibodies. 25 µg of normal (wt) and transgenic (tg) mouse brain extracts were analyzed by SDS-PAGE as indicated. M, molecular weight marker (kDa). Tg mHTT, transgenic human polyglutamine-expanded HTT. WT HTT, endogenous mouse wild type HTT.