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SDHA (11C2) Rabbit Monoclonal Antibody  Cite:0    分享

货号： IM68950
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  • 种属：H，M，R
  • 用途：WB，ELISA，IHC-P，IF-P，IF-F，IF-ICC
规格:
50μl 100μl
单位:
支
单价:￥1300.00
产品简介
储存与保存
注意事项
产品简介
  • 产品货号
    IM68950
  • 别名
    SDHA;SDH2;SDHF;Succinate dehydrogenase [ubiquinone] flavoprotein subunit; mitochondrial;Flavoprotein subunit of complex II;Fp
  • 产品名称
    SDHA (11C2) Rabbit Monoclonal Antibody
  • 类别
    抗体产品
  • 基因名称
    SDHA
  • 蛋白名称
    Succinate dehydrogenase [ubiquinone] flavoprotein subunit mitochondrial
  • Clonality
    Monoclonal
  • 推荐应用
    WB,IHC-P,IF-P,IF-F,IF-ICC,ELISA
  • 反应种属
    Human,Mouse,Rat
  • 存储缓冲液
    PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
  • Human Gene ID
    6389
  • Human Swissprot No.
    P31040
  • Mouse Gene ID
    66945
  • Mouse Swissprot No.
    Q8K2B3
  • Rat Gene ID
    157074
  • Rat Swissprot No.
    Q920L2
  • 稀释度
    IHC-P 1:200-1000, WB 1:1000-5000, IF-P/IF-F/IF-ICC 1:200-1000, ELISA 1:5000-20000
  • 参考分子量
    73kDa
  • 预测分子量
    73kDa
  • 宿主
    Rabbit
  • 同种型
    Monoclonal, rabbit, IgG, Kappa
  • 背景介绍
    This gene encodes a major catalytic subunit of succinate-ubiquinone oxidoreductase, a complex of the mitochondrial respiratory chain. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. Mutations in this gene have been associated with a form of mitochondrial respiratory chain deficiency known as Leigh Syndrome. A pseudogene has been identified on chromosome 3q29. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jun 2014],
  • 信号通路
    Citrate cycle (TCA cycle);Oxidative phosphorylation;Alzheimer's disease;Parkinson's disease;Huntington's disease.
  • 功能
    catalytic activity:Succinate + ubiquinone = fumarate + ubiquinol.,cofactor:FAD.,disease:Defects in SDHA are a cause of complex II mitochondrial respiratory chain deficiency [MIM:252011]; also known as succinate CoQ reductase deficiency. Defects of oxidative phosphorylation give rise to heterogeneous clinical symptoms ranging from isolated organ dysfunction to multisystem disorder. A deficiency of complex II represents a rare cause of mitochondrial encephalomyopathy, leukodystrophy, late-onset optic atrophy and ataxia, myopathy with exercise intolerance, and isolated cardiomyopathy.,disease:Defects in SDHA are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,function:Flavoprotein (FP) 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).,miscellaneous:The complex, present in mitochondria, can be degraded to form EC 1.3.99.1, which no longer reacts with ubiquinone.,pathway:Carbohydrate metabolism; tricarboxylic acid cycle.,sequence caution:Differs extensively from that shown.,similarity:Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily.,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.
  • 纯化
    Protein A
储存与保存

1.保存：-20℃

2.有效期：1年

注意事项

1.我司生产的生化试剂如无特殊标注，基本为非无菌包装，若用于细胞实验，请提前做好预处理。需低温保存的产品，一旦配成溶液，请分装保存，避免反复冻融造成的产品失效。

2.本产品仅供科研使用。请勿用于医药、临床诊断或治疗，食品及化妆品等用途。请勿存放于普通住宅区。 

3.为了您的安全和健康，请穿好实验服并佩戴一次性手套和口罩操作。 

4.实验结果可由多种因素影响，相关处理只限于产品本身，不涉及其他赔偿。 

 

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