Animal ID:
RB2011/2012
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Host:
Rabbit
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Reactivity:
human
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Buffers:
Purified rabbit polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column and eluted out with both high and low pH buffers and neutralized immediately after elution then followed by dialysis against PBS.
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Storage:
Maintain refrigerated at 2-8ºC for up to 6 months. For long term storage store at -20ºC. Avoid repeated freeze-thaw cycles.
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Application:
Tested by peptide-specific ELISA (1:1,000).
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Immunogen:
KLH conjugated synthetic peptide comprised of amino acids 639 - 653 [YRRQLRWKMLQKGEC] of the human huntingtin-associated protein 1 (HAP1) protein.
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Accession number:
NM_003949
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Description:
Huntington's disease (HD) is caused by the expansion of a glutamine repeat in the protein huntingtin. The expanded glutamine repeat is thought to mediate a gain of function by causing huntingtin to abnormally interact with other proteins. As vitro binding, immunoprecipitation, and co-expression studies have shown, the huntingtin-associated protein 1 (HAP1) physically interacts with huntingtin. Human HAP1 is a 75 kDa protein which is specifically expressed in human brain and binds more tightly to mutant huntingtin with an expanded glutamine repeat than to wild type huntingtin. Human HAP1 shares 62% identity with rat HAP1 over its entire sequence and 82% amino acid identity in the putative huntingtin-binding region. Its expression in Huntington's disease brains is reduced in parallel with a decreased expression of huntingtin.
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Alternative Name(s):
huntingtin-associated protein 1, huntingtin-associated protein 2, neuroan 1, HLP, HAP2, HIP5, hHLP1,
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References:
- Nasir J, Lafuente MJ, Duan K, Colomer V, Engelender S, Ingersoll R, Margolis RL, Ross CA, Hayden MR: Human huntingtin-associated protein (HAP-1) gene: genomic organisation and an intragenic polymorphism. Gene 254(1-2): 181-187 (2000).
- Nasir J, Maclean A, Engelender S, Duan K, Margolis RL, Kleiderlein JJ, Ross CA, Hayden MR: Chromosomal localization of the Huntingtin associated protein (HAP-1) gene in mouse and humans with radiation hybrid and interspecific backcross mapping. Mamm. Genome 10(4): 397-398 (1999).
- Li SH, Hosseini SH, Gutekunst CA, Hersch SM, Ferrante RJ, Li XJ: A human HAP1 homologue. Cloning, expression, and interaction with huntingtin. J. Biol. Chem 273(30): 19220-19227 (1998).
- Engelender S, Sharp AH, Colomer V, Tokito MK, Lanahan A, Worley P, Holzbaur EL, Ross CA: Huntingtin-associated protein 1 (HAP1) interacts with the p150Glued subunit of dynactin. Hum. Mol. Genet. 6(13): 2205-2212 (1997).
- Li XJ, Li SH, Sharp AH, Nucifora FC Jr, Schilling G, Lanahan A, Worley P, Snyder SH, Ross CA: A huntingtin-associated protein enriched in brain with implications for pathology. Nature 378(6555): 398-402 (1995).
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For Research Use Only. Not for Diagnostic or Therapeutic Use.
Purchase does not include or carry any right to resell or transfer this product either as a stand-alone product or as a component of another product. Any use of this product other than the permitted use without the express written authorization of Orbigen, Inc. is strictly prohibited
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