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SDHA抗体经ICC, IHC严格验证,品质保证.适用于多种种属反应性,被多篇文献引用并有用户反馈信息.确保特异性!产物具有以下特点:

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产物编号xy-3970R

英文名称厂顿贬础

中文名称琥珀酸脱氢酶复合体亚基础抗体

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.  

说 明 书100ul  200ul

研究领域肿瘤  细胞生物  **学  信号转导  细胞凋亡  转录调节因子  

抗体来源搁补产产颈迟

克隆类型笔辞濒测肠濒辞苍补濒

SDHA抗体交叉反应 Human, Mouse, Rat,

产物应用WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.

分 子 量70kDa

性    状Lyophilized or Liquid

浓    度1mg/1ml

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.

PubMedPubMed

产物介绍产补肠办驳谤辞耻苍诲:

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.

SWISS:

P31040

Gene ID:

6389

Important Note:

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