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顶部产品介绍抗原信息化学信息储存和溶解信息计算器

Anti-SDHA Polyclonal Antibody

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货号 TMAB-12602

Anti-SDHA Polyclonal Antibody 是一种 Rabbit 抗体，靶向 SDHA。Anti-SDHA Polyclonal Antibody 可用于 IHC-P,IHC-Fr,IF,FCM。

Anti-SDHA Polyclonal Antibody

Anti-SDHA Polyclonal Antibody

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Rating icon 还可以
货号 TMAB-12602

Anti-SDHA Polyclonal Antibody 是一种 Rabbit 抗体，靶向 SDHA。Anti-SDHA Polyclonal Antibody 可用于 IHC-P,IHC-Fr,IF,FCM。

规格价格库存数量
50 μL
¥ 1,160
5日内发货
100 μL
¥ 1,960
5日内发货
200 μL
¥ 2,795
5日内发货
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产品介绍

生物活性
产品描述
Anti-SDHA Polyclonal Antibody is a Rabbit antibody targeting SDHA. Anti-SDHA Polyclonal Antibody can be used in IHC-P,IHC-Fr,IF,FCM.
Ig Type
IgG
反应种属
Human,Mouse (predicted:Rat)
应用IHC-PIHC-FrIFFCM
推荐剂量
IHC-P: 1:100-500; IHC-Fr: 1:100-500; IF: 1:100-500; FCM: 2μg/Test
抗体种类
Polyclonal
宿主来源Rabbit
构建方式Hybridoma Polyclonal Antibody
纯化方式Protein A purified
性状Liquid
缓冲液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
浓度1mg/ml
研究背景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.
抗原信息
免疫原
KLH conjugated synthetic peptide: human SDHA
抗原种属
Human
基因名称
SDHA
基因ID
6389
蛋白名称
Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
Uniprot ID
P31040
功能
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.
化学信息
分子量Theoretical: 70 kDa.
储存&溶解度
储存方式Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles.
运输方式Shipping with blue ice.
存储store at low temperature | -20°C for 1 year

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