Recombinant human NDUFS2 protein
NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform 1 precursor, also known as NDUFS2, is a 463 amino acid protein that is suggested to be required for catalytic activity. Defects in NDuFS2 are the cause of complex I mitochondrial respiratory chain deficiency, which is characterized by a variety of symptoms including liver failure, cardiomyopathy and neurodegeneration. Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. Recombinant human NDUFS2 protein, fused to His-tag at N-terminus, was expressed in E. coli.
Product Specifications
Product Name Alternative
Expression System
E.coli
Antigen Species
Human
Tag
His-Tag
Applications
SDS-PAGE, Denatured
Concentration
0.25 mg/mL (determined by Bradford assay)
Purity
> 80% by SDS-PAGE
Molecular Weight
46.5 kDa (410aa)
Additionnal Information
References & Citations
Loeffen J., et al. (2001) Ann Neurol. 49:195-201.; ; Procaccio V., et al. (1998) Genome. 9:482-484.;
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 0.4M urea, 10% glycerol
Scientific Category
Metabolism
NCBI Accession Number
NP_004541
Uniprot Accession Number
O75306
Species
Human
AA Sequence
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