Huntingtin (human), pAb



BML-PW0595-0025	25 µl	92.00 USD

BML-PW0595-0100	100 µl	325.00 USD

Product Specification

Alternative Name:	Htt, HD Protein

Purity Detail:	Purified by salt precipitation.

Formulation:	Liquid. In PBS containing 0.01% sodium azide.

Immunogen:	Synthetic peptide corresponding to aa 2-17 of human huntingtin.

Source/Host:	From rabbit.

Specificity:	Recognizes human huntingtin (irrespective of SUMOylation status).

Application:	Western Blot (1:1'000) Optimal conditions must be determined individually for each application.

Short Term Storage:	+4°C

Long Term Storage:	-20°C

Use/Stability:	Stable for at least 1 year after receipt when stored at -20°C. Dilute with PBS, pH 7.2-7.4, containing 1% normal goat serum (if a goat anti-rabbit IgG linker antibody is to be used). Store diluted antibody at +4°C, do not freeze and use within 1 month.

Handling:	After opening, prepare aliquots and store at -20°C. Avoid freeze/thaw cycles.

Miscellaneous/General:	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.

Figure: Western blot detection of Htt exon 1 (A) and SUMO-Htt exon1 (B) in transfected HeLa cell lysates using PAb to Huntingtin (human) (Prod. No. BML-PW0595) at 1:1\'000 dilution

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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;

SUMO modification of Huntingtin and Huntington’s disease pathology: J.S. Steffan, et al.; Science 304, 100 (2004), 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;