产品货号 :  mlR17294 

英文名称 :  SCO1

中文名称 :  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.   

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 :  Human, Mouse, Rat, Cow, Horse, Rabbit, Sheep, 

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user. 

分 子 量 :  34kDa 

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid 

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human SCO1:201-301/301 

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. 

PubMed :  PubMed

产品介绍 :  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.

SWISS:

O75880

Gene ID:

603644 

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.