产品基本信息

• 产品货号 RM8278
• 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 Gene Link http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=6389
• Human Swissprot No. P31040
• Human Swissprot Link http://www.uniprot.org/uniprotkb/P31040/entry
• Mouse Gene ID 66945
• Mouse Gene Link http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=66945
• Mouse Swissprot No. Q8K2B3
• Mouse Swissprot Link http://www.uniprot.org/uniprot/Q8K2B3
• Rat Gene ID 157074
• Rat Gene Link http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=157074
• Rat Swissprot No. Q920L2
• Rat Swissprot Link http://www.uniprot.org/uniprot/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
• 运输及保存条件 -20°C/1 year(Do not lower than -25°C)
• 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

图片

	Western blot analysis of lysates from Hela cells, using SDHA Rabbit mAb. The HRP-conjugated Goat anti-Rabbit IgG antibody was used to detect the antibody.
	Immunohistochemical analysis of paraffin-embedded Human kidney tissue. 1, primary Antibody was diluted at 1:200(4℃,overnight). 2, EDTA pH 9.0 was used for antibody retrieval(>98℃,20min). 3,Secondary antibody was diluted at 1:200(room tempeRature, 30min).
	Immunohistochemical analysis of paraffin-embedded Rat kidney tissue. 1, primary Antibody was diluted at 1:200(4℃,overnight). 2, EDTA pH 9.0 was used for antibody retrieval(>98℃,20min). 3,Secondary antibody was diluted at 1:200(room tempeRature, 30min).

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