上海细胞库
人源细胞系| 稳转细胞系| 基因敲除株| 基因点突变细胞株| 基因过表达细胞株| 重组细胞系| 猪的细胞系| 马细胞系| 兔的细胞系| 犬的细胞系| 山羊的细胞系| 鱼的细胞系| 猴的细胞系| 仓鼠的细胞系| 狗的细胞系| 牛的细胞| 大鼠细胞系| 小鼠细胞系| 其他细胞系|
人源细胞系| 稳转细胞系| 基因敲除株| 基因点突变细胞株| 基因过表达细胞株| 重组细胞系| 猪的细胞系| 马细胞系| 兔的细胞系| 犬的细胞系| 山羊的细胞系| 鱼的细胞系| 猴的细胞系| 仓鼠的细胞系| 狗的细胞系| 牛的细胞| 大鼠细胞系| 小鼠细胞系| 其他细胞系|
| 规格 | 价格 | 库存 |
|---|---|---|
| 50ul | ￥ 1200 | 7 |
| 100ul | ￥ 1900 | 5 |
| 200ul | ￥ 2900 | 3 |
| 产品编号 | Ys-3970R |
| 英文名称 | SDHA |
| 中文名称 | 琥珀酸脱氢酶复合体亚基A抗体 |
| 别 名 | mitochondrial; DHSA_HUMAN; Flavoprotein subunit of complex II; Fp; SDH 2; SDH2; SDHA; SDHF; Succinate dehydrogenase [ubiquinone] flavoprotein subunit; Succinate dehydrogenase [ubiquinone] flavoprotein subunit mitochondrial; Succinate dehydrogenase complex flavoprotein subunit precursor; Succinate dehydrogenase complex subunit A; Succinate Dehydrogenase Complex subunit A Flavoprotein. |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, (predicted: Rat, ) |
| 产品应用 | WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=2ug/Test IF=1:100-500 （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. |
| 理论分子量 | 70kDa |
| 细胞定位 | 细胞浆 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SDHA: 561-664/664 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 | In aerobic respiration reactions, succinate dehydrogenase (SDH) catalyzes the oxidation of succinate and ubiquinone to fumarate and ubiquinol. Four subunits comprise the SDH protein complex: a flavochrome subunit (SDHA), an iron-sulfur protein (SDHB), and two membrane-bound subunits (SDHC and SDHD) anchored to the inner mitochondrial membrane. Mutations to these subunits cause mitochondrial dysfunction, corresponding to several distinct disorders. Mutations in the membrane bound components may cause hereditary paraganglioma, while SDHA mutations are associated with juvenile encephalopathy as well as Leigh Syndrome, a severe neurological disorder. Inactivating mutations in SDHB correlate with inherited, but not necessarily sporadic, cases of pheochromocytoma. Function: Defects in SDHA are a cause of mitochondrial complex II deficiency (MT-C2D). A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome. |
