UBE3A (C-term) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	ELISA: 1/1,000. Western Blot: 1/100-1/500. Immunohistochemistry: 1/50-1/100.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the C-terminal region of human UBE3A.
Specificity	This antibody is specific to UBE3A (C-term).
Formulation	PBS with 0.09% (W/V) Sodium Azide as preservative. State: Purified State: Liquid purified Ig fraction.
Concentration	lot specific
Purification	Protein G Chromatography, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Conjugation	Unconjugated
Storage Condition	Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Gene Name	ubiquitin protein ligase E3A
Database Link	Entrez Gene 7337 Human UniProt ID Q05086
Background	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.
Synonyms	Ubiquitin-protein ligase E3A, E6AP, EPVE6AP, HPVE6A, NY-REN-54
Note	Predicted Molecular weight: 100645 Da
Reference Data

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