Description
Ubiquitin-protein ligase E3A Antibody / UBE3A | F54766-0.4ML | Gentaur US, UK & Europe Disrtribition
Family: Primary antibody
Formulation: In 1X PBS, pH 7.4, with 0.09% sodium azide
Format: Purified
Clone: N/A
Host Animal: Rabbit
Clonality: Polyclonal (rabbit origin)
Species Reactivity: Human
Application: FACS, IF, IHC-P, WB
Buffer: N/A
Limitation: This Ubiquitin-protein ligase E3A antibody is available for research use only.
Purity: Purified
Description: 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.
Immunogen: A portion of amino acids 836-865 from the human protein was used as the immunogen for the Ubiquitin-protein ligase E3A antibody.
Storage: Aliquot the Ubiquitin-protein ligase E3A antibody and store frozen at -20 °C or colder. Avoid repeated freeze-thaw cycles.
