BD-PB3990

UBE3A Rabbit Polyclonal Antibody (C-term)

Ubiquitin-protein ligase E3A, 632-, E6AP ubiquitin-protein ligase, Human papillomavirus E6-associated protein, Oncogenic protein-associated protein E6-AP, Renal carcinoma antigen NY-REN-54, UBE3A, E6AP, EPVE6AP, HPVE6A

常规抗体

WB

Mouse, Human

NP_000453.2;NP_570853.1;NP_570854.1

Q05086

This UBE3A antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 836-865 amino acids from the C-terminal region of human UBE3A.

WB~~1:1000;IHC-P~~1:100~500;IF~~1:10~50;FC~~1:10~50

101kDa

Rabbit IgG

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.

Cytoplasm {ECO:0000250|UniProtKB:O08759}. Nucleus {ECO:0000250|UniProtKB:O08759}

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