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Rabbit Anti-SCO1/Gold Conjugated antibody (bs-17294R-Gold)
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-17294R-Gold
英文名称 Anti-SCO1/Gold
中文名称 胶体金标记的细胞色素氧化酶缺失蛋白1抗体
别    名 Cytochrome oxidase deficient homolog; Cytochrome oxidase deficient homolog 1; Protein SCO1 homolog mitochondrial; Protein SCO1 homolog, mitochondrial; SCO (cytochrome oxidase deficient yeast) homolog 1; SCO cytochrome oxidase deficient homolog 1 (yeast); SCO cytochrome oxidase deficient homolog 1; sco1; SCO1_HUMAN; SCOD1.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
研究领域 肿瘤  心血管  细胞生物  免疫学  神经生物学  细胞类型标志物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Cow, Horse, Rabbit, Sheep, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 34kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCO1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 background:
Mammalian cytochrome c oxidase (COX) catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. In yeast, 2 related COX assembly genes, SCO1 and SCO2 (synthesis of cytochrome c oxidase), enable subunits 1 and 2 to be incorporated into the holoprotein. This gene is the human homolog to the yeast SCO1 gene. [provided by RefSeq, Jul 2008]

Function:
Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX.

Subcellular Location:
Mitochondrion.

Tissue Specificity:
Predominantly expressed in tissues characterized by high rates of oxidative phosphorylation (OxPhos), including muscle, heart, and brain.

DISEASE:
Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.

Similarity:
Belongs to the SCO1/2 family.

Database links:

Entrez Gene: 6341 Human

Entrez Gene: 52892 Mouse

Entrez Gene: 497930 Rat

Omim: 603644 Human

SwissProt: O75880 Human

SwissProt: Q5SUC9 Mouse

Unigene: 14511 Human

Unigene: 129731 Mouse

Unigene: 473182 Mouse

Unigene: 203819 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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