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Rabbit Anti-SURF1/Cy5 Conjugated antibody (bs-12846R-Cy5)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-12846R-Cy5
英文名称 Anti-SURF1/Cy5
中文名称 Cy5标记的过量位点蛋白1抗体
别    名 OTTHUMP00000022473; OTTHUMP00000022474; SURF 1; SURF-1; Surf1; SURF1_HUMAN; Surfeit 1; Surfeit locus protein 1.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  神经生物学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Horse, )
产品应用 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    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SURF1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 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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