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Rabbit Anti-SURF1/BF488 Conjugated antibody (bs-12846R-BF488)
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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-12846R-BF488
英文名稱1 Rabbit Anti-SURF1/BF488 Conjugated antibody
中文名稱 BF488標(biāo)記的過(guò)量位點(diǎn)蛋白1抗體
別    名 OTTHUMP00000022473; OTTHUMP00000022474; SURF 1; SURF-1; Surf1; SURF1_HUMAN; Surfeit 1; Surfeit locus protein 1.  
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  細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Horse, )
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 33, 80 and 116kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SURF1
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:
This gene encodes a protein localized to the inner mitochondrial membrane and thought to be involved in the biogenesis of the cytochrome c oxidase complex. The protein is a member of the SURF1 family, which includes the related yeast protein SHY1 and rickettsial protein RP733. The gene is located in the surfeit gene cluster, a group of very tightly linked genes that do not share sequence similarity, where it shares a bidirectional promoter with SURF2 on the opposite strand. Defects in this gene are a cause of Leigh syndrome, a severe neurological disorder that is commonly associated with systemic cytochrome c oxidase deficiency. [provided by RefSeq, Jul 2008]

Function:
Probably involved in the biogenesis of the COX complex.

Subcellular Location:
Mitochondrion inner membrane.

DISEASE:
Defects in SURF1 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency.

Similarity:
Belongs to the SURF1 family.

Database links:

Entrez Gene: 6834 Human

Entrez Gene: 20930 Mouse

Entrez Gene: 64463 Rat

Omim: 185620 Human

SwissProt: Q15526 Human

SwissProt: P09925 Mouse

SwissProt: Q9QXU2 Rat

Unigene: 512464 Human

Unigene: 347512 Mouse

Unigene: 74313 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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