Cat. No.
MABL-2715
Application
WB, IHC
Isotype
Engineer antibody
Species Reactivity
Human
Clone No.
MW1
From
Recombinant Antibody
Specificity
This antibody binds the extended polyglutamine repeats (GQQQQQQQQQQG) of the mutant huntingtin exon 1. It does not bind the wild type Htt.
Alternative Names
PolyQ; HTT; htt; Huntington disease protein; HD protein; huntingtin; Glutamine repeats; Atrophin-1
UniProt
P42858
Immunogen
This antibody was raised against a 53 amino acid fragment of atrophin-1 containing poly Q (19) and purified from ascites fluid by protein G affinity chromatography.
Application Notes
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).
Antibody First Published
Ko et al. New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins. Brain Research Bulletin (2001); Volume 56; Issues 3–4; Pages 319-329 PMID:11719267
Note on publication
Describes the generation and characterization of the antibody.
Size
100 μg Purified antibody.
Concentration
1 mg/ml.
Purification
Protein A affinity purified
Buffer
PBS with 0.02% Proclin 300.
Storage Recommendation
Store at 4⁰C for up to 3 months. For longer storage, aliquot and store at - 20⁰C.
