Recombinant UBE3A Monoclonal Antibody

SKU: AN302038L

Size::50μL

Storage:Store at -20℃ Valid for 12 months. Avoid freeze / thaw cycles.

Shipping:Ice bag

Exp date:12 months

Category ID_II:Primary Antibodies

Category ID_III:Monoclonal Antibodies;Recombinant Antibodies

Abbreviation:UBE3A

Target Synonym:UBE3A;ANCR;AS;E6-AP;EPVE6AP;HPVE6A;E6AP;ubiquitin protein ligase E3A

Research Areas:Epigenetics and Nuclear Signaling;Cell Biology;Neuroscience

Conjugation:Unconjugated

Host:Rabbit

Species reactivity:Human;

Application:WB

Isotype:IgG,κ

Clonality:Monoclonal;Recombinant

Clone NO.:A758

UNIProt ID:Q05086

Accession:

Background:UBE3A, also commonly referred to as E6AP (E6 Associated Protein), is an E3 ubiquitin protein ligase and founding member of the HECT (Homologous to the E6 Carboxyl Terminus) family of E3 ligases. UBE3A has been shown to be hijacked by the oncogenic E6 protein of high-risk human papillomaviruses (HPV16 and HPV18) that causes the ubiquitination activity of UBE3A to be inappropriately directed toward several specific cellular proteins, the most notable of which, with respect to carcinogenesis, is p53. Although the DNA-repair enzyme, HHR23A (human homolog A of Rad23), was the first described E6-independent substrate of UBE3A, very few E6-independent targets of UBE3A have been identified. This continues to be an active area of research, particularly because mutations or disruption in expression of UBE3A in the brain are the cause of Angelman syndrome (AS), a severe form of mental retardation. Although UBE3A is expressed in most human tissues from both parental alleles, it is expressed from the maternal allele in subregions of the brain, with the paternal allele being epigenetically silenced. AS is caused by disruptions in expression of the materal UBE3A allele, generally by large chromosomal deletion, but also by point mutations within the UBE3A coding sequence. This strongly suggests that lack of ubiquitination of one or more UBE3A substrates in neuronal tissue is responsible for the AS phenotype. Indeed, a recent study identified several new neuronal substrates of UBE3A including Arc and Ephexin-5. The immediate early gene Arc (activity-regulated cytoskeleton-associated protein) is rapidly upregulated after robust neuronal stimulation and promotes internalization of AMPA-type glutamate receptors (AMPARs), resulting in reduction in synaptic strength. UBE3A ubiquitinates Arc and promotes its degradation by the 26S proteasome, thus preventing AMPAR internalization. Disruption in neuronal UBE3A function leads to an increase in Arc expression and a decrease in AMPARs at excitatory synapses, which may contribute to the neurological symptoms of AS.

Concentration:1 mg/mL

Immunogen:Peptide. This information is proprietary to PTMab.

Buffer:PBS, 50% glycerol, 0.05% Proclin 300, 0.05% protein protectant.

Purification method:Protein A purified

Dilution:WB 1:5000

Calculated MW:101 kDa

ObservedMW:95 kDa