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Rabbit Anti-IKAP/BF488 Conjugated antibody (bs-13644R-BF488)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-13644R-BF488
英文名稱 Rabbit Anti-IKAP/BF488 Conjugated antibody
中文名稱 BF488標(biāo)記的IKK復(fù)合物相關(guān)蛋白抗體
別    名 DKFZp781H1425; DYS; Dysautonomia (Riley Day syndrome hereditary sensory autonomic neuropathy type III); Elongator complex protein 1; ELP 1; ELP1; ELP1_HUMAN; FD; FLJ12497; IKAP; IkappaB kinase complex associated protein; IkappaB kinase complex-associated protein; ikbkap; IKI 3; IKI3; IKK complex associated protein; IKK complex-associated protein; Inhibitor of kappa light polypeptide gene enhancer in B cells kinase complex associated protein; OTTHUMP00000063889; p150; TOT 1; TOT1  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 心血管  細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  激酶和磷酸酶  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Rabbit, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 150kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human IKAP
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 transcription factor NFkB is retained in the cytoplasm in an inactive form by the inhibitory protein IkB. Activation of NFkB requires that IkB be phosphorylated on specific serine residues, which results in the targeted degradation of IkB (1). IkB kinase alpha (IKK alpha), previously designated CHUK (2), interacts with IkB-alpha and specifically phosphorylates IkB-alpha on the sites that trigger its degradation, serines 32 and 36 (3). IKKalpha appears to be critical for NFkB activation in response to proinflammatory cytokines (4). Phosphorylation of the IkB by IKK alpha is stimulated by the NFkB inducing kinase (NIK), which itself is a central regulator for NFkB activation in response to TNF and IL-1 (5). The functional IKK complex contains three subunits, IKK alpha, IKK beta and IKK gamma (also designated NEMO), and each appears to make essential contributions to IkB phosphorylation (6). IKAP (IKK-complex-associated protein) is a protein that acts as a scaffold, interacting with NIK, IKK alpha and IKK beta and assembling them into an active kinase complex (7,8)

Function:
May act as a scaffold protein that may assemble active IKK-MAP3K14 complexes (IKKA, IKKB and MAP3K14/NIK).
Acts as subunit of the RNA polymerase II elongator complex, which is a histone acetyltransferase component of the RNA polymerase II (Pol II) holoenzyme and is involved in transcriptional elongation. Elongator may play a role in chromatin remodeling and is involved in acetylation of histones H3 and probably H4.

Subcellular Location:
Cytoplasm. Nucleus.

DISEASE:
Defects in IKBKAP are the cause of hereditary sensory and autonomic neuropathy type 3 (HSAN3) [MIM:223900]; also known as Riley-Day syndrome or familial dysautonomia (FD). This autosomal recessive disorder is due to the poor development and survival, and progressive degeneration of the sensory, sympathetic and parasympathetic neurons. HSAN3 individuals are affected with a variety of symptoms such as decreased sensitivity to pain and temperature, cardiovascular instability, recurrent pneumonias, vomiting crises, and gastrointestinal dysfunction. It is primarily confined to individuals of Ashkenazi Jewish descent, with an incidence of 1/3'600 live births.

Similarity:
Belongs to the ELP1/IKA1 family.

Database links:

Entrez Gene: 8518 Human

Entrez Gene: 230233 Mouse

Entrez Gene: 100009360 Rabbit

Entrez Gene: 140934 Rat

Omim: 603722 Human

SwissProt: O95163 Human

SwissProt: Q7TT37 Mouse

SwissProt: Q8WND5 Rabbit



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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