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Rabbit Anti-VCP/BF647 Conjugated antibody (bs-1443R-BF647)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-1443R-BF647
英文名稱 Rabbit Anti-VCP/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的含纈酪肽蛋白抗體
別    名 valosin-containing protein; 15S Mg(2+) ATPase p97 subunit; ATPase p97; IBMPFD; MGC131997; MGC148092; MGC8560; p97; TER ATPase; TERA; transitional endoplasmic reticulum ATPase; valosin-containing protein; VCP; yeast Cdc48p homolog; Transitional endoplasmic reticulum ATPase; TER ATPase; 15S Mg(2+)-ATPase p97 subunit; p97.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 腫瘤  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  生長(zhǎng)因子和激素  糖尿病  糖蛋白  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Cow, Horse, )
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 97kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human VCP
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 is a member of a family that includes putative ATP-binding proteins involved in vesicle transport and fusion, 26S proteasome function, and assembly of peroxisomes. This protein, as a structural protein, is associated with clathrin, and heat-shock protein Hsc70, to form a complex. It has been implicated in a number of cellular events that are regulated during mitosis, including homotypic membrane fusion, spindle pole body function, and ubiquitin-dependent protein degradation. [provided by RefSeq, Jul 2008]

Function:
Necessary for the fragmentation of Golgi stacks during mitosis and for their reassembly after mitosis. Involved in the formation of the transitional endoplasmic reticulum (tER). The transfer of membranes from the endoplasmic reticulum to the Golgi apparatus occurs via 50-70 nm transition vesicles which derive from part-rough, part-smooth transitional elements of the endoplasmic reticulum (tER). Vesicle budding from the tER is an ATP-dependent process. The ternary complex containing UFD1L, VCP and NPLOC4 binds ubiquitinated proteins and is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome. The NPLOC4-UFD1L-VCP complex regulates spindle disassembly at the end of mitosis and is necessary for the formation of a closed nuclear envelope (By similarity). Regulates E3 ubiquitin-protein ligase activity of RNF19A.

Subcellular Location:
Cytoplasm > cytosol. Nucleus. Present in the neuronal hyaline inclusion bodies specifically found in motor neurons from amyotrophic lateral sclerosis patients. Present in the Lewy bodies specifically found in neurons from Parkinson disease patients.

Post-translational modifications:
Phosphorylated by tyrosine kinases in response to T-cell antigen receptor activation (By similarity).
Phosphorylated upon DNA damage, probably by ATM or ATR. ISGylated.

DISEASE:
Defects in VCP are the cause of inclusion body myopathy with early-onset Paget disease and frontotemporal dementia (IBMPFD) [MIM:167320]; also known as muscular dystrophy, limb-girdle, with Paget disease of bone or pagetoid amyotrophic lateral sclerosis or pagetoid neuroskeletal syndrome or lower motor neuron degeneration with Paget-like bone disease. IBMPFD features adult-onset proximal and distal muscle weakness (clinically resembling limb girdle muscular dystrophy), early-onset Paget disease of bone in most cases and premature frontotemporal dementia.

Similarity:
Belongs to the AAA ATPase family.

Database links:

Entrez Gene: 7415 Human

Entrez Gene: 269523 Mouse

Entrez Gene: 116643 Rat

Omim: 601023 Human

SwissProt: P55072 Human

SwissProt: Q01853 Mouse

SwissProt: P46462 Rat

Unigene: 529782 Human

Unigene: 245976 Mouse

Unigene: 98891 Rat



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

含纈酪肽蛋白(VCP即p97),是一種廣泛存在的膜結(jié)合糖蛋白,在細(xì)胞活性中有著廣泛的功能,其特點(diǎn)是作為類似分子伴侶的作用在內(nèi)質(zhì)網(wǎng)相關(guān)的蛋白降解及細(xì)胞周期調(diào)控中起到重要作用,VCP蛋白可以和肝素在細(xì)胞表面結(jié)合,而起到保護(hù)細(xì)胞的作用。
目前對(duì)VCP的表達(dá)水平與食道癌、胃癌、結(jié)、直腸癌、肝癌、胰腺癌等消化系統(tǒng)惡性腫瘤等是研究的熱點(diǎn)。
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