泛素激活酶E1抗体
规格：1mg/1ml
英文名： E1 Ubiquitin Activating Enzyme
别名： A1S9; A1S9 protein; A1S9T and BN75 temperature sensitivity complementing; A1S9T; A1ST; GXP 1; GXP1; MGC4781; Protein A1S9; Uba1; UBA1_HUMAN; UBE 1 ; UBE 1X; UBE1; UBE1X; Ubiquitin activating enzyme E1
分子量： 118kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human UBE1
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：
泛素激活酶E1抗体产品介绍：background: The protein encoded by this gene catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. This gene complements an X-linked mouse temperature-sensitive defect in DNA synthesis, and thus may function in DNA repair. It is part of a gene cluster on chromosome Xp11.23. Alternatively spliced transcript variants that encode the same protein have been described. [provided by RefSeq, Jul 2008]. Function: Activates ubiquitin by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding an ubiquitin-E1 thioester and free AMP. Subunit: Monomer (By similarity). Interacts with GAN (via BTB domain). Post-translational modifications: ISGylated. DISEASE: Defects in UBA1 are the cause of spinal muscular atrophy X-linked type 2 (SMAX2) [MIM:301830]; also known as X-linked lethal infantile spinal muscular atrophy, distal X-linked arthrogryposis multiplex 泛素激活酶E1抗体congenita or X-linked arthrogryposis type 1 (AMCX1). Spinal muscular atrophy refers to a group of neuromuscular disorders characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. SMAX2 is a lethal infantile form presenting with hypotonia, areflexia, and multiple congenital contractures. Similarity: Belongs to the ubiquitin-activating E1 family. Gene ID: 7317 Database links: Entrez Gene: 7317 Human Entrez Gene: 22201 Mouse Entrez Gene: 314432 Rat Omim: 314370 Human SwissProt: 泛素激活酶E1抗体P22314 Human SwissProt: Q02053 Mouse SwissProt: Q5U300 Rat Unigene: 533273 Human Unigene: 1104 Mouse Unigene: 474674 Mouse Unigene: 11800 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
泛素激活酶E1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 神经生物学 信号转导 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid