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Rabbit Anti-SDHA/APC Conjugated antibody (bs-3970R-APC)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-3970R-APC
英文名称1 Rabbit Anti-SDHA/APC Conjugated antibody
中文名称 APC标记的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.  
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  信号转导  细胞凋亡  转录调节因子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse,  (predicted: Rat, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 70kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SDHA
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:
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.

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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