线粒体复合物NDUFS1蛋白抗体
规格：1mg/1ml
英文名： NDUFS1
别名： Complex I 75Kd; Complex I, mitochondrial respiratory chain, 75 kD subunit; NADH coenzyme Q reductase; NADH dehydrogenase (ubiquinone) FeS protein 1 (75kD) (NADH coenzyme Q reductase) ; NADH ubiquinone
分子量： 79kDa
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 Ndufs1
交叉反应：Human, Mouse, Rat, Dog, Pig, Rabbit,
细胞定位：细胞浆 细胞膜 线粒体
线粒体复合物NDUFS1蛋白抗体产品介绍：background: Ndufs1 belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. Ndufs1 has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in Ndufs1 gene are associated with complex I deficiency. 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. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the线粒体复合物NDUFS1蛋白抗体 enzyme. It may form part of the active site crevice where NADH is oxidized. Subcellular Location: Mitochondrion inner membrane. Similarity: Defects in NDUFS1 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to *****-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, 线粒体复合物NDUFS1蛋白抗体Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
线粒体复合物NDUFS1蛋白抗体产品应用：WB=1:100-500 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 心血管 细胞生物 **学 神经生物学 结合蛋白 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