Cell Signaling Technology

Product Pathways - Protein Stability

UBE3A (D10D3) Rabbit mAb #7526

No. Size Price
7526S 100 µl ( 10 western blots ) ¥3,100.00 现货查询 购买询价
7526 carrier free & custom formulation / quantityemail request
Applications Dilution Species-Reactivity Sensitivity MW (kDa) Isotype
W 1:1000 Human,Mouse,Rat,Monkey, Endogenous 98 Rabbit IgG

Species cross-reactivity is determined by western blot.

Applications Key: W=Western Blotting,

Homology

Species predicted to react based on 100% sequence homology: Bovine, Dog, Horse,

Specificity / Sensitivity

UBE3A (D10D3)兔单抗识别内源性的UBE3A总蛋白。根据蛋白序列比对预测这个抗体可以识别所有UBE3A剪接体。

Source / Purification

UBE3A (D10D3)兔单抗是通过合成对应于人UBE3A蛋白羧基末端序列的肽段,免疫动物获得。 Western blot analysis of extracts from various cell lines using UBE3A (D10D3) Rabbit mAb. 使用UBE3A (D10D3)兔单抗对不同细胞裂解物进行免疫印迹分析的结果。 Western blot analysis of extracts from 293T cells, either mock transfected (-) or transfected with a cDNA expression construct encoding transcript variant 3 of human UBE3A (hUBE3A, +), using UBE3A (D10D3) Rabbit mAb. 使用UBE3A (D10D3)兔单抗对转染对照载体(-)或转染含有人UBE3A基因转录本3序列的表达载体(+)的293T细胞裂解物进行免疫印迹分析的结果。

Western Blotting

Western Blotting

Western blot analysis of extracts from various cell lines using UBE3A (D10D3) Rabbit mAb.

Western Blotting

Western Blotting

Western blot analysis of extracts from 293T cells, either mock transfected (-) or transfected with a cDNA expression construct encoding transcript variant 3 of human UBE3A (hUBE3A, +), using UBE3A (D10D3) Rabbit mAb.

Background

UBE3A,也通常被称作E6AP (E6相关蛋白6),是HECT(Homologous to the E6 Carboxyl Terminus)E3泛素连接酶家族的成员(1)。早期研究表明,UBE3A被高致病性人乳头瘤病毒的癌基因产物E6蛋白所利用,会造成UBE3A针对几个特异的细胞蛋白进行异常的泛素化修饰,其中最为重要的靶蛋白就是p53(2)。尽管DNA损伤修复酶HHR23A(human homolog A of Rad23)是第一个被发现的E6非依赖性UBE3A底物,但被发现的其他E6非依赖性UBE3A底物很少。这一领域后来成为一个非常活跃的研究领域,部分原因是由于脑部UBE3A基因突变或者表达缺失会导致一种严重的神经退行性疾病——Angelman综合症(AS)的发生。尽管UBE3A可以在多数人体组织中由两个等位基因座进行表达,但其在脑中由于父本基因座的印记失活作用,主要由母本基因座表达。Angelman综合症的发生,主要是由于大范围染色体缺失造成母本UBE3A基因座表达缺失,同时还伴有UBE3A编码区域的点突变。这一证据提示,脑组织中一个或多个UBE3A底物泛素化修饰的缺失会造成Angelman综合症的表型(7)。实际上,最近的一个报道鉴定了几个UBE3A在脑组织的新底物,包括Arc和Ephexin-5 (8)。立即早期基因Arc (activity-regulated cytoskeleton-associated protein)在强烈的神经刺激后表达迅速升高,并促进AMPA类型的谷氨酸受体(AMPARs)内化过程,导致突触传导能力的减退。UBE3A泛素化修饰Arc,促进其通过26S蛋白酶体降解,这就避免了AMPAR的内化(8)。神经组织UBE3A功能的受损会导致在兴奋性突触中Arc表达升高和AMPARs的减少,这是Angelman综合症的发生的可能机制。

  1. Huibregtse, J.M. et al. (1995) Proc Natl Acad Sci U S A 92, 5249.
  2. Huibregtse, J.M. et al. (1993) Mol Cell Biol 13, 775-84.
  3. Fang, P. et al. (1999) Hum Mol Genet 8, 129-35.
  4. Jiang, Y. et al. (1999) Am J Hum Genet 65, 1-6.
  5. Jiang, Y.H. et al. (1998) Neuron 21, 799-811.
  6. Kumar, S. et al. (1999) J Biol Chem 274, 18785-92.
  7. Mabb, A.M. et al. (2011) Trends Neurosci 34, 293-303.
  8. Greer, P.L. et al. (2010) Cell 140, 704-16.

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