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货号： bs-3934R-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-3934R-Cy7
- 英文名称
- Anti-COX3/Cy7
- 中文名称
- Cy7标记的细胞色素C氧化酶亚基3抗体
- 别 名
- Cytochrome c oxidase polypeptide III; Cytochrome c oxidase subunit 3; COIII; COX3; COX-3; COXIII; MT CO3; MTCO3; COX3_HUMAN; mitochondrially encoded cytochrome c oxidase III; MT-CO3.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 免疫学 神经生物学 信号转导 转录调节因子 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
- 产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 30kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human COX3
- 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.
- 产品介绍
- Function:
Subunits I, II and III form the functional core of the enzyme complex.
Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein.
DISEASE:
Leber hereditary optic neuropathy (LHON) [MIM:535000]: A maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Note=The disease is caused by mutations affecting the gene represented in this entry.
Mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]: 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, exercise intolerance, developmental delay, delayed motor development and mental retardation. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome. Note=The disease is caused by mutations affecting the gene represented in this entry.
Recurrent myoglobinuria mitochondrial (RM-MT) [MIM:550500]: Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine. Note=The gene represented in this entry may be involved in disease pathogenesis.
Similarity:
Belongs to the cytochrome c oxidase subunit 3 family.
Database links:Entrez Gene: 281921 Cow
Entrez Gene: 4514 Human
Entrez Gene: 17710 Mouse
Entrez Gene: 26204 Rat
Omim: 516050 Human
SwissProt: P00415 Cow
SwissProt: P00414 Human
SwissProt: P00416 Mouse
SwissProt: P05505 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.