The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
HIRA interacting protein 5
HIRA-interacting protein 5
Histone cell cycle regulation defective interacting protein 5
Iron sulfur cluster scaffold protein
NFU1 iron sulfur cluster scaffold homolog
NFU1 iron sulfur cluster scaffold homolog (S. cerevisiae)
NFU1 iron sulfur cluster scaffold homolog, mitochondrial
NFU1 iron-sulfur cluster scaffold homolog
Iron-sulfur cluster scaffold protein which can assemble [4Fe-2S] clusters and deliver them to target proteins.
Ubiquitous. Expression in adult lung is weak compared to fetal lung.
Involvement in disease
Defects in NFU1 are the cause of multiple mitochondrial dysfunctions syndrome type 1 (MMDS1) [MIM:605711]. A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes.