细胞色素氧化酶缺失蛋白1抗体
规格：1mg/1ml
英文名： SCO1
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
分子量： 34kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human SCO1
交叉反应：Human, Mouse, Rat,
细胞定位：
细胞色素氧化酶缺失蛋白1抗体产品介绍：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 细胞色素氧化酶缺失蛋白1抗体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 细胞色素氧化酶缺失蛋白1抗体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.
细胞色素氧化酶缺失蛋白1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域：肿瘤 心血管 细胞生物 **学 神经生物学 细胞类型标志物
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid