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货号： bs-8530R-AF488 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-8530R-AF488
- 英文名称
- Anti-SDHC/AF488
- 中文名称
- AF488标记的琥珀酸细胞色素亚基B560抗体
- 别 名
- mitochondrial; QPs1; C560_HUMAN; CYBL; Integral membrane protein CII-3; QPs-1; sdhC; Succinate dehydrogenase complex subunit C; Succinate dehydrogenase cytochrome b560 subunit; Succinate dehydrogenase cytochrome b560 subunit, mitochondrial precursor; Succinate-ubiquinone oxidoreductase cytochrome B large subunit.
- 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 发育生物学 神经生物学 信号转导 表观遗传学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human,
- 产品应用
- 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
- 15kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Succinate dehydrogenase complex subunit C/SDHC
- 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
- background:
Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone.
Function:
Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Subunit:
Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD.
Subcellular Location:
Mitochondrion inner membrane.
DISEASE:
Defects in SDHC are the cause of paragangliomas type 3 (PGL3) [MIM:605373]. A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas are most commonly located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear.
Defects in SDHC are a cause of paraganglioma and gastric stromal sarcoma (PGGSS) [MIM:606864]; also known as Carney-Stratakis syndrome. Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance.
Similarity:
Belongs to the cytochrome b560 family.
Database links:Entrez Gene: 327696Cow
Entrez Gene: 6391Human
Entrez Gene: 66052Mouse
Entrez Gene: 100524676Pig
Entrez Gene: 289217Rat
Omim: 602413Human
SwissProt: P35720Cow
SwissProt: Q99643Human
SwissProt: Q9CZB0Mouse
SwissProt: D0VWV4Pig
Unigene: 444472Human
Unigene: 198138Mouse
Unigene: 1698Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Involvement in disease:Defects in SDHC are the cause of hereditary paragangliomas type 3 (PGL3) [MIM:605373]; also known as autosomal dominant non-chromaffin paragangliomas type 3. Non-chromaffin paragangliomas are usually benign, neural crest derived tumors of parasympathetic ganglia.
Defects in SDHC are a cause of paraganglioma and gastric stromal sarcoma (PGGSS) ; also known as Carney-Stratakis syndrome. Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance.