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, a Huntingtin monoclonal antibody (Product # MA1-41172). B. An Htt antibody recognizing both normal and mutant Htt (B). a Huntingtin monoclonal antibody (Product # MA1-41172) recognized only the expanded ~65 polyQ repeat form of Htt (CAG-L), showing no detectable binding to the normal ~20 repeat Htt form (CAG-S). In contrast, the other antibody recognized both the expanded (~65) and normal (~20) repeat Htt forms.
|Tested species reactivity||Human, Mouse|
|Host / Isotype||Mouse / IgG|
|Immunogen||Fusion protein containing a repeating sequence of 62 consecutive glutamine residues.|
|Contains||0.05% sodium azide|
|Storage Conditions||Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.|
|Tested Applications||Dilution *|
|Western Blot (WB)||1 µg/ml|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
Clone 1A771 binds to the expanded polyQ repeat form of Htt in WB, showing no detectable binding to normal Htt in WB.
Suggested positive control: Lymphoblast cell line extracts from HD patients.
Huntington's disease (HD) is a neurodegenerative disorder caused by an expanding polyglutamine repeat in the huntingtin 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 is widely expressed in human tissues, with the highest level of expression in the brain. The huntington gene product is expressed at similar levels in 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 yeast two-hybrid system Li et al., have identified a protein, HAP1 (huntington associated protein 1), that associates with huntingtin protein. The in vitro data suggest that the association between HAP1 and huntington is enhanced by increasing length of glutamine repeat.
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