Anti-Polyglutamine [MW1]

Absolute Antibody

Anti-Polyglutamine [MW1]

9

This antibody binds the extended polyglutamine repeats (GQQQQQQQQQQG) of the mutant huntingtin exon 1. It does not bind the wild type Htt.

This antibody strongly prefers to bind the expanded polyQ repeat forms of the mutant huntingtin and displayed no detectable binding to normal huntingtin in Western blot assay. These results suggested that the polyQ domain can assume different conformations that are distinquishable by mAbs. Immunohistochemistry with wild type (WT) and mutant Htt transgenic mouse (R6) brains shows that different antibodies have different staining patterns. MW1 shows punctate cytoplasmic and neuropil staining in immunohistochemical staining (PMID: 11719267). When MW1 scFv is co-transfected in 293 cells with htt exon 1 containing an expanded polyQ domain, it stimulate htt aggregation and apoptosis (PMID: 11792860). The crystal structure of polyQ bound to MW1, reveals that polyQ adopts an extended, coil-like structure. Consistent with the linear lattice model, multimeric MW1 Fvs bind more tightly to longer than to shorter polyQ tracts and, compared with monomeric Fv, bind expanded polyQ repeats with higher apparent affinities (PMID: 17450152).

Western Blot, ImmunoHistoChemistry

Research Use Only

Monoclonal

MW1

HTT

huntingtin

HD; huntingtin; huntington disease protein; IT15; LOMARS

Mouse

Huntingtin

Human

-20°C,2°C to 8°C

12352203