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Rabbit Anti-SURF1  antibody (bs-12846R)
~~~促销,代码KX240301~~~
~~~促销,代码KX240302~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-12846R
英文名称 SURF1
中文名称 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.  
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 IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 33, 80 and 116kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SURF1: 131-230/300 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

SWISS:
Q15526

Gene ID:
6834

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



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