琥珀酸细胞色素亚基B560抗体
规格:1mg/1ml
英文名: SDHC
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
分子量: 15kDa
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 Succinat
交叉反应:Human,
细胞定位:细胞浆 细胞膜
产琥珀酸细胞色素亚基B560抗体品介绍: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: 327696 Cow Entrez Gene: 6391 Human Entrez Gene: 66052 Mouse Entrez Gene: 100524676 Pig Entrez Gene: 289217 Rat Omim: 602413 Human SwissProt: P35720 Cow SwissProt琥珀酸细胞色素亚基B560抗体: Q99643 Human SwissProt: Q9CZB0 Mouse SwissProt: D0VWV4 Pig Unigene: 444472 Human Unigene: 198138 Mouse Unigene: 1698 Rat 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琥珀酸细胞色素亚基B560抗体. 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.
琥珀酸细胞色素亚基B560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=1:50-200 (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 细胞生物 发育生物学 神经生物学 信号转导 表观遗传学
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid