Immunofluorescent analysis of HeLa cells using a UBE3A polyclonal antibody (Product # PA5-12038) at a dilution of 1:10-50, followed by a fluor-conjugated goat anti-rabbit secondary antibody (green). Actin filaments were stained with dye-conjugated phalloidin (red). Nuclei were stained with DAPI (blue).
|Tested species reactivity||Human|
|Host / Isotype||Rabbit / IgG|
|Immunogen||KLH conjugated synthetic peptide between 836-865 amino acids from the C-terminal region of human UBE3A|
|Purification||Size-exclusion - Dialysis, Ammonium sulfate precipitation|
|Contains||0.09% sodium azide|
|Storage Conditions||-20° C, Avoid Freeze/Thaw Cycles|
|Tested Applications||Dilution *|
|Flow Cytometry (Flow)||1:10-1:50|
|Immunohistochemistry (Paraffin) (IHC (P))||1:50-1:100|
|Western Blot (WB)||1:1000|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
UBE3A interacts with the E6 protein of the cancer-associated human papillomavirus types 16 and 18. The E6/E6-AP complex binds to and targets the p53 tumor-suppressor protein for ubiquitin-mediated proteolysis. It is an E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and then directly transfers the ubiquitin to targeted substrates. It can target itself for ubiquitination in vitro and efficiently promotes its own degradation in vivo. It appears that only unmodified E6-AP molecules can bind efficiently to p53 in the presence of the HPV E6 oncoprotein. UBE3A binds UBQLN1 and UBQLN2. Defects in UBE3A are a cause of Angelman syndrome (AS) [MIM:105830]; also known as 'happy puppet syndrome'. AS is characterized by features of severe motor and intellectual retardation, microcephaly, ataxia, frequent jerky limb movements and flapping of the arms and hands, hypotonia, hyperactivity, hypopigmentation, seizures, absence of speech, frequent smiling and episodes of paroxysmal laughter, and an unusual facies characterized by macrostomia, a large mandible and open-mouthed expression, a great propensity for protruding the tongue ('tongue thrusting'), and an occipital groove. UBE3A contains 1 HECT-type E3 ubiquitin-protein ligase domain.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.