Huntingtin Protein (Huntington Disease)

Huntington's disease (HD) is a neurodegenerative disorder caused by an expanding polyglutamine repeat in the huntingtin gene(1). HD is a mid-life onset autosomal dominant neurodegeneative disease that is characterized by psychiatric disorders, dementia, and involuntary movements (chorea), leading to death in 10-20 years2,3. The HD gene product is widely expressed in human tissues, with the highest level of expression in the brain1,4,5. The huntingtin 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 (huntingtin associated protein 1), that associates with huntingtin protein8. The In vitro data suggest that the association between HAP1 and huntingtin is enhanced by increasing length of glutamine repeat. \n\nApplications: \nSuitable for use in Western Blot. Other applications have not been tested. \n\nRecommended Dilutions: \nWestern Blot: 1ug/ml\nOptimal dilutions to be determined by the researcher. \n\nRecommended Positive Control:\nLymphoblast cell line extracts\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

H7965-01A

Size

100ug

Applications

WB

Hosts

Mouse

Reactivities

Hum, Mouse

Form

Supplied as a liquid in PBS, pH 7.2, 0.2% gelatin, 0.05% sodium azide.

P Type

Mab

Purity

Purified by Protein G affinity chromatography.

Isotype

IgG

References

1. Huntington's Disease Collaborative Research Group. Cell 72: 971-983 (1993). 2. Fostein SE. Huntington's Disease: A disorder of Families, The John Hopkins Universiy Press. 3. Martin JB & Gusella JF. New Engl. J. Med. 315: 1267-1276 (1986). 4. Strong TV, Tagle DA, Valles JM, Elmer CW, Boehm K, Swaroop M, Kaatz KW, Collins FS, and Albin RL. Nature Genet 5: 259-265 (1993). 5. Sharp AH, Loev SJ, Schilling G, Li SH, Li XJ, Bao J, Wagster MV, Kotzuk JA, Steiner JP, Lo A, et al. Neuron 14 (5): 1065-1074 (1995). 6. Albin RL and Tagle DA. Trends Neurosci 18: 11-14 (1995). 7. Ross CA. Neuron 15: 493-496 (1995). 8. Li X-J, Li S-H, Sharp AH, Nucifora Jr. FC, Schilling G, Lanaha A, Worley P, Snyder SH, Ross CA. Nature 378: 398-402 (1995).

Additional Info

Recognizes human Huntingtin Protein. \nSpecies Crossreactivity: mouse.