| 產(chǎn)品編號(hào) | bs-17294R-BF647 |
| 英文名稱1 | Rabbit Anti-SCO1/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的細(xì)胞色素氧化酶缺失蛋白1抗體 |
| 別 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. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 心血管 細(xì)胞生物 免疫學(xué) 神經(jīng)生物學(xué) 細(xì)胞類型標(biāo)志物 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Cow, Horse, Rabbit, Sheep, ) |
| 產(chǎn)品應(yīng)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 | 34kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SCO1 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
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 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. |
