Anti-UBE3A Picoband® Antibody (monoclonal, 8I3)

SKU: M00582-2

Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Monkey

Applications: WB,Flow Cytometry

Application Details: Western blot, 0.25-0.5μg/ml, Human, Monkey
Flow Cytometry (Fixed), 1-3μg/1x10⁶ cells, Human

Gene Name: UBE3A

Specificity:

Background: Ubiquitin-protein ligase E3A (UBE3A) also known as E6AP ubiquitin-protein ligase (E6AP) is an enzyme that in humans is encoded by the UBE3A gene. It is mapped to 15q11.2. This gene encodes an E3 ubiquitin-protein ligase, part of the ubiquitin protein degradation system. This imprinted gene is maternally expressed in brain and biallelically expressed in other tissues. Maternally inherited deletion of this gene 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. Alternative splicing of this gene results in three transcript variants encoding three isoforms with different N-termini. Additional transcript variants have been described, but their full length nature has not been determined.

Immunogen: E.coli-derived human UBE3A recombinant protein (Position: M1-E860).

Clonality: Monoclonal

Contents: Each vial contains 4mg Trehalose, 0.9mg NaCl and 0.2mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Abaied, L., Trabelsi, M., Chaabouni, M., Kharrat, M., Kraoua, L., M'rad, R., Tebib, N., Maazoul, F., Chaabouni, H. A novel UBE3A truncating mutation in large Tunisian Angelman syndrome pedigree. Am. J. Med. Genet. 152A: 141-146, 2010. 2. Albrecht, U., Sutcliffe, J. S., Cattanach, B. M., Beechey, C. V., Armstrong, D., Eichele, G., Beaudet, A. L. Imprinted expression of the murine Angelman syndrome gene, Ube3a, in hippocampal and Purkinje neurons. Nature Genet. 17: 75-78, 1997. 3. Camprubi, C., Guitart, M., Gabau, E., Coll, M. D., Villatoro, S., Oltra, S., Rosello, M., Ferrer, I., Monfort, S., Orellana, C., Martinez, F. Novel UBE3A mutations causing Angelman syndrome: different parental origin for single nucleotide changes and multiple nucleotide deletions or insertions. Am. J. Med. Genet. 149A: 343-348, 2009.

Uniprot ID: Q05086

Host: Mouse

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Mouse IgG2a

Phospho_site:

Clone Number: Clone: 8I3

Observed Molecular Weight: 100 kDa

Calculated Molecular Weight:

Gene ID: 7337

Protein Name: Ubiquitin-protein ligase E3A

Gene Full Name: ubiquitin protein ligase E3A

Synonyms: ANCR; AS; E6 AP; E6AP; E6AP ubiquitin protein ligase; EPVE6AP; HPVE6A; UBE3A; ubiquitin protein ligase E3A