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Rabbit Anti-E1 Ubiquitin Activating Enzyme/BF350 Conjugated antibody (bs-6835R-BF350)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-6835R-BF350
英文名稱(chēng) Rabbit Anti-E1 Ubiquitin Activating Enzyme/BF350 Conjugated antibody
中文名稱(chēng) BF350標(biāo)記的泛素激活酶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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
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(lèi)型 Polyclonal
交叉反應(yīng) Human, Rat,  (predicted: Mouse, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 118kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human UBE1
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 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 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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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