NADH脱氢酶黄素蛋白1抗体
规格:1mg/1ml
英文名: NDUFV1
别名: CI 51kD; Complex I 51kD; NADH dehydrogenase (ubiquinone) flavoprotein 1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; NADH dehydrogenase flavoprotein 1; NADH ubiquinone oxidoreductas
分子量: 49kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human NDUFV1
交叉反应:Human, Mouse, Rat, Dog, Pig, Cow, Horse,
细胞定位:细胞浆 细胞膜 线粒体
NADH脱氢酶黄素蛋白1抗体产品介绍:background: The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009] Function: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. TheNADH脱氢酶黄素蛋白1抗体 immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). Subunit: Complex I is composed of 45 different subunits. This is a component of the flavoprotein-sulfur (FP) fragment of the enzyme. Subcellular Location: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. DISEASE: Defects in NDUFV1 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Similarity: Belongs to the complex I 51 kDa subunit family. Gene ID: 4723 Database links: Entrez Gene: 287014 Cow Entrez Gene: 4723 Human Entrez Gene: 17995 Mouse Entrez Gene: 293655 Rat OmimNADH脱氢酶黄素蛋白1抗体 161015 Human SwissProt: P25708 Cow SwissProt: P49821 Human SwissProt: Q91YT0 Mouse SwissProt: Q5XIH3 Rat Unigene: 7744 Human Unigene: 29842 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
NADH脱氢酶黄素蛋白1抗体产品应用:ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域:肿瘤 细胞生物 **学 线粒体
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid