NDUFS2抗体
[别名:NDUS2_HUMAN; NADH dehydrogenase [ubiquinone: iron-sulfur protein 2, mitochondrial; Complex I-49kD; CI-49kD; NADH-ubiquinone oxidoreductase 49 kDa subunit; NADH dehydrogenase [ubiquinone: iron-sulfur protein 2, mitochondrial isoform 1 precursor; CI-49.
[产品介绍:NDUFS2抗体The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[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. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Subunit : Complex I is composed of 45 different subunits. Component of the iron-sulfur (IP) fragment of the enzyme. Interacts with NDUFAF3.
Subcellular Location : Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
DISEASE : Defects in NDUFS2 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 adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
Similarity : Belongs to the complex I 49 kDa subunit family.
Database links : UniProtKB/Swiss-Prot: O75306.2
规格:多种抗体规格提供
贮存: 贮存于-20℃
纯化方法:亲和纯化的蛋白
研究领域:细胞生物 免疫学 锌指蛋白
产品应用比例:WB=1:100-500 Elisa=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1/20-1/100
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