Replaces Prod. #: BML-PW0595
Product Details
Alternative Name: | Htt, HD Protein |
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Host: | Rabbit |
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Immunogen: | Synthetic peptide corresponding to aa 2-17 of human huntingtin. |
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UniProt ID: | P42858 |
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Source: | Purified from rabbit serum. |
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Species reactivity: | Mouse, Rat
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Applications: | WB
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Recommended Dilutions/Conditions: | Western Blot (1:1,000) Suggested dilutions/conditions may not be available for all applications. Optimal conditions must be determined individually for each application. |
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Application Notes: | Detects a band of ~350kDa by Western blot. |
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Purity Detail: | Protein A affinity purified. |
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Formulation: | Liquid. In PBS containing 50% glycerol and 0.09% sodium azide. |
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Handling: | Avoid freeze/thaw cycles. After opening, prepare aliquots and store at -20°C. |
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Shipping: | Blue Ice |
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Short Term Storage: | +4°C |
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Long Term Storage: | -20°C |
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Scientific Background: | Huntington’s disease (HD) is a neurodegenerative disorder caused by an expanding polyglutamine repeat in the huntingtin gene (>35 repeats results in HD onset). Expansion of this polyglutamine repeat may induce a toxic gain of function perhaps through interactions with other cellular proteins. The huntingtin protein is thought to be necessary for neuronal survival and is involved in synaptic vesicle trafficking, microtubule binding and may also have a role in apoptosis. SUMO modification at the N-terminus of the huntingtin protein is also believed to play a role in the neurodegenerative process. |
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Regulatory Status: | RUO - Research Use Only |
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Western blot analysis of Huntingtin pAb (Prod. No. BML-PW0595A): Lane 1: MW Marker, Lane 2: Mouse Brain Tissue Extract (Prod. No. ADI-LYT-MB100), Lane 3: Rat Brain Tissue Extract (Prod. No. ADI-LYT-RB100).
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General Literature References
Global changes to the ubiquitin system in Huntington’s disease: E.J. Bennett, et al.; Nature
448, 704 (2007),
Abstract;
Huntington’s disease: the challenge for cell biologists: A.J. Tobin & E.R. Signer; Trends Cell Biol.
10, 531 (2000),
Abstract;
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. The Huntington’s Disease Collaborative Research Group: Cell
72, 971 (1993),
Abstract;
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