Animal ID:
RB0153-0154
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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 83 - 100 [CDPHPSKKGASSAYLENS] of the human ubiquitin protein ligase E3A (UBE3A) protein.
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Accession number:
NM_000462
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Description:
The ubiquitin protein ligase E3A (UBE3A) is part of the ubiquitin protein degradation system. The UBE3A gene is imprinted and only maternally expressed in brain and biallelically expressed in other tissues. Maternally inherited deletion of UBE3A causes Angelman Syndrome, characterized by severe motor and intellectual retardation, ataxia, hypotonia, epilepsy, absence of speech, and characteristic facies. The protein also interacts with the E6 protein of human papillomavirus types 16 and 18, resulting in ubiquitination and proteolysis of tumor protein p53. The UBE3A gene encodes 3 different protein isoforms (1, 2 and 3). UBE3A protein isoform 2 is the longest and isoform 1 uses a downstream in-frame start codon resulting in a 23 amino acid shorter protein than isoform 2. Isoform 3 includes amino acids at the N-terminus which are encoded by three unique exons as compared to variant 1 and 2. All three isoforms are recognized by this antibody.
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Alternative Name(s):
AS, ANCR, E6-AP, EPVE6AP, CTCL tumor antigen se37-2, oncogenic protein-associated protein E6AP, human papilloma virus E6-associated protein
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References:
- Wong EY, Tse JY, Yao KM, Tam PC, Yeung WS: VCY2 protein interacts with the HECT domain of ubiquitin-protein ligase E3A. Biochem. Biophys. Res. Commun. 296(5): 1104-1111 (2002).
- Herzing LB, Cook EH Jr, Ledbetter DH: Allele-specific expression analysis by RNA-FISH demonstrates preferential maternal expression of UBE3A and imprint maintenance within 15q11- q13 duplications. Hum. Mol. Genet. 11(15): 1707-1718 (2002).
- Lossie AC, Whitney MM, Amidon D, Dong HJ, Chen P, Theriaque D, Hutson A, Nicholls RD, Zori RT, Williams CA, Driscoll DJ: Distinct phenotypes distinguish the molecular classes of Angelman syndrome. J. Med. Genet. 38(12): 834-845 (2001).
- Kishino T, Lalande M, Wagstaff J: UBE3A/E6-AP mutations cause Angelman syndrome. Nat. Genet. 15(1): 70-73 (1997).
- Huibregtse JM, Scheffner M, Howley PM: Cloning and expression of the cDNA for E6-AP, a protein that mediates the interaction of the human papillomavirus E6 oncoprotein with p53. Mol. Cell. Biol. 13(2): 775-784 (1993).
- Scheffner M, Huibregtse JM, Vierstra RD, Howley PM: The HPV-16 E6 and E6-AP complex functions as a ubiquitin-protein ligase in the ubiquitination of p53. Cell 75(3): 495-505 (1993).
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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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