Product Name	Anti-SCO1/RBITC
Chinese Name	罗丹明（RBITC）标记的细胞色素氧化酶缺失蛋白1抗体
Alias	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.
Research Area	Tumour  Cardiovascular  Cell biology  immunology  Neurobiology  Cell type markers
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Cow, Horse, Rabbit, Sheep, )
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	34kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human SCO1
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	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.
Product Detail	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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