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E1 Ubiquitin Activating Enzyme Rabbit pAb (bs-6835R)  
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產(chǎn)品編號(hào) bs-6835R
英文名稱 E1 Ubiquitin Activating Enzyme Rabbit pAb
中文名稱 泛素激活酶E1抗體
別    名 Uba1; A1S9; A1S9 protein; A1S9T and BN75 temperature sensitivity complementing; A1S9T; A1ST; GXP 1; GXP1; MGC4781; Protein A1S9; UBA1_HUMAN; UBE 1; UBE 1X; UBE1; UBE1X; Ubiquitin activating enzyme E1; Ubiquitin-activating enzyme E1; Ubiquitin-like modifier-activating enzyme 1.  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  表觀遺傳學(xué)  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
克 隆 號(hào)
交叉反應(yīng) Human,Rat (predicted: Mouse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 118 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞核 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human UBE1: 961-1058/1058 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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 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.

SWISS:
P22314

Gene ID:
7317

Database links:

Entrez Gene: 7317 Human

Entrez Gene: 22201 Mouse

Entrez Gene: 314432 Rat

Omim: 314370 Human

SwissProt: P22314 Human

SwissProt: Q02053 Mouse

SwissProt: Q5U300 Rat

Unigene: 533273 Human

Unigene: 1104 Mouse

Unigene: 474674 Mouse

Unigene: 11800 Rat



產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (E1 Ubiquitin Activating Enzyme) Polyclonal Antibody, Unconjugated (bs-6835R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.
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