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折扣/货期| 货号：GTX85056 | 规格：100μg | 目录价：￥6200 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
SCO1 antibody
别名:
SCO cytochrome c oxidase assembly protein 1 , SCOD1
反应种属:
Human, Mouse, Rat
宿主来源:
Rabbit
实验应用:
ELISA, IHC-P, WB
同种型:
IgG
免疫原:
SCO1 antibody was raised against a 14 amino acid synthetic peptide from near the center of human SCO1.The immunogen is located within amino acids 100 - 150 of SCO1.
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by antigen-affinity chromatography
偶联:
Unconjugated
产品浓度:
1 mg/ml (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
预期分子量:
34
产品形式:
Liquid
存储溶液:
PBS, 0.02% Sodium azide.
生产商:
GeneTex
功能与背景:
Synthesis of cytochrome c oxidase 1 was initially identified in yeast as one of two cytochrome c oxidase (COX) assembly proteins that enable the assembly of cytochrome c holoenzyme, a complex that catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. Like their yeast homologs, the function of both SCO1 and SCO2 are dependent on copper ion binding. Mutations in either gene can lead to cytochrome c oxidase respiratory chain defects, with a missense mutation in human SCO1 (P174L) associated with a fatal neonatal hepatopathy when the second allele is also non-functional, suggesting the pathology is due to loss of function. It has been suggested that this mutation alters the SCO1 affinity for the copper (I) ion, thus impairing the efficiency of copper transfer to the cytochrome c oxidase. At least two isoforms of SCO1 are known to exist and both are recognized by the SCO1 antibody. This SCO1 antibody has no cross-reactivity to SCO2.
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