Catalog # HM4841

Huntingtin, mutant-specific Antibody

Mouse Monoclonal

Application / Dilution

1:500

Size 100 μl

Species Reactivity Hu, Ms

MW 350 kDa

Isotype IgG1

Huntington's disease (HD) is a neurodegenerative disorder caused by an expanding polyglutamine repeat in the huntingtin (Htt) gene. HD is a mid-life onset autosomal dominant neurodegenerative disease that is characterized by psychiatric disorders, dementia, and involuntary movements (chorea), leading to death in 10-20 years. The HD gene product HTT is widely expressed in human tissues, with the highest level of expression in the brain. Furthermore, HTT is expressed at similar levels in HD patients and controls, which suggests that the expansion of the polyglutamine repeat induces a toxic gain of function perhaps through interactions with other cellular proteins. Using a yeast two-hybrid approach, HAP1 (huntington associated protein 1) was identified. HAP1 associates with HTT. The in vitro data suggest that the association between HAP1 and HTT is enhanced by increasing length of the glutamine repeat in HTT.

References

Sharp A.H. et al. (1995) Neuron. 14(5):1065.
Albin R.L. & Tagle D.A. (1995) Trends Neurosci. 18:11.
Strong T.V. et al. (1993) Nature Genet. 5:259.

Western blot analysis of Htt in four different lymphoblast HD cell lines extracts each expressing ~65 (mutant) and ~20 (normal) CAG repeats. Lanes 1-4. A) Anti-HTT mutant-specific antibody HM4841. B) An Htt antibody recognizing both normal and mutant Htt. HM4841 recognized only the expanded ~65 polyQ repeat form of Htt (CAG-L) with no detectable binding to the normal ~20 repeat Htt form (CAG-S).

Clone 1A771 was generated from Huntingtin mutant protein containing 62 glutamine amino acid repeats.

*For more information, see UniProt Accession P42858

Mouse monoclonal antibody purified with protein G chromatography is supplied in 100μl phosphate-buffered saline with 0.2% gelatin and 0.05% sodium azide. Store at 4°C, stable for 6 months. For long term storage, aliquot and store at -20°C.

The products are are safely shipped at ambient temperature for both domestic and international shipments. Each product is guaranteed to match the specifications as indicated on the corresponding technical data sheet. Please store at -20C upon arrival for long term storage.

This antibody detects expanded polyQ repeat form of Htt in western blot, and does not detect normal Htt in a lymphoblast cell line from HD patients.

*All molecular weights (MW) are confirmed by comparison to Bio-Rad Rainbow Markers and to western blot mobilities of known proteins with similar MW.

This kit contains: