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品种反应性 主要应用 宿主 格式 抗体类型 M, H ICC, WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 ABT110 描述 Anti-SDHD (CybS) Antibody Alternate Names - Succinate-ubiquinone reductase membrane anchor subunit
- Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
- CybS
- CII-4
- QPs3
- Succinate dehydrogenase complex subunit D
- Succinate-ubiquinone oxidoreductase cytochrome b small subunit
背景信息 Succinate dehydrogenase complex subunit D (SDHD) is a member of the CybS family and is one of four subunits of the succinate dehydrogenase (SDH) complex involved in the electron transport chain of the mitochondria. The specific role of SDH is to transfer electrons from succinate to ubiquinone, and the SDHD subunit is involved in anchoring the SDH complex to the inner mitochondrial membrane. Defective SDHD has been linked to Carney-Stratakis syndrome, a condition characterized by concurrent paraganglioma and gastric stromal sarcomas. 产品信息 格式 Affinity Purified 控制 - HeLa cell lysate.
演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 This Anti-SDHD (CybS) Antibody is validated for use in Western Blotting, ICC for the detection of SDHD (CybS). 主要应用 - Immunocytochemistry
- Western Blotting
应用说明 Immunocytochemistry Analysis: A 1:500 dilution from a representative lot detected SDHD (CybS) in NIH/3T3 and HeLa cells. 生物信息 免疫原品种 KLH-conjugated linear peptide corresponding to the Topological domain of human SDHD (CybS). 表位 Topological domain (mitochondrial matrix) 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 特异性 This antibody recognizes the Topological domain (mitochondrial matrix) of SDHD (CybS). 品种反应性 MouseHuman Species Reactivity Note Demonstrated to react with Human and Mouse. Predicted to react with Bovine, Sheep, and Rhesus Macaque based on 100% sequence homology. Other homologies: Rat (92% sequence homology). 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_002993
Entrez基因汇总 Complex II of the respiratory chain, which is specifically involved in the oxidation of succinate, carries electrons from FADH to CoQ. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. The subunit D protein is one of two integral membrane proteins anchoring the complex to the matrix side of the membrane. Mutations in SDHD have been linked to hereditary paraganglioma. 基因符号 - SDH4
- SDHD
纯化方法 Affinity purified UniProt编号 - O14521
UniProt汇总 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).
PATHWAY: Carbohydrate metabolism; tricarboxylic acid cycle.
SUBUNIT STRUCTURE: 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; Multi-pass membrane protein.
INVOLVEMENT IN DISEASE: Defects in SDHD are a cause of paragangliomas type 1 (PGL1). A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. PGL1 is a rare autosomal dominant disorder which is characterized by the development of mostly benign, highly vascular, slowly growing tumors in the head and neck. In the head and neck region, the carotid body is the largest of all paraganglia and is also the most common site of the tumors. Defects in SDHD are a cause of susceptibility to pheochromocytoma (PCC) [MIM:171300]. A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent. Defects in SDHD may be a cause of susceptibility to intestinal carcinoid tumor (ICT). A yellow, well-differentiated, circumscribed tumor that arises from enterochromaffin cells in the small intestine or, less frequently, in other parts of the gastrointestinal tract. Defects in SDHD are a cause of paraganglioma and gastric stromal sarcoma (PGGSS); also called 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. Defects in SDHD are a cause of Cowden-like syndrome (CWDLS). Cowden-like syndrome is a cancer predisposition syndrome associated with elevated risk for tumors of the breast, thyroid, kidney and uterus.
SEQUENCE SIMILARITIES: Belongs to the CybS family.产品使用声明 质量保证 Evaluated by Western Blot in HeLa cell lysate.
Western Blot Analysis: 1 µg/mL of this antibody detected SDHD (CybS) in 10 µg of HeLa cell lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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