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.

Target	UBE3A
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Tested Applications	WB, IHC, IF/ICC, FCM
Recommended dilutions	Optimal dilutions/concentrations should be determined by the end user.
Immunogen	KLH-conjugated synthetic peptide between 836-865 amino acids from the C-terminal region of human UBE3A.
Purification	Purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Isotype	IgG
Conjugation	Unconjugated
Storage	Aliquot and store at -20 °C. Avoid repeated freeze/thaw cycles.
Swiss Prot	Q05086
Buffer	PBS containing 0.09% sodium azide.
UNSPSC Code	12352203
Availability	Shipped within 5-10 working days.
Note	This product is for research use only.