Anti-NFU1 Antibody

Raised in rabbit against the iron-sulfur cluster scaffold protein NFU1, this unconjugated polyclonal primary antibody from Antibodies.com is supplied at 1 mg/ml in PBS containing 0.1% sodium azide and 50% glycerol as a 100 µl aliquot, ready for direct use in standard immunodetection workflows. NFU1 functions in mitochondrial [2Fe-2S] and [4Fe-4S] cluster biogenesis, accepting nascent clusters from the ISCU scaffold and delivering them to mitochondrial recipient enzymes, and biallelic mutations in the gene are linked to multiple mitochondrial dysfunctions syndrome type 1 (MMDS1) and fatal cavitating leukodystrophy, which makes the reagent a useful tool for studies of mitochondrial iron-sulfur assembly, oxidative phosphorylation, and related metabolic disease models. The antibody has been validated for Western blot (WB) and immunohistochemistry (IHC) applications and shows reactivity with Human, Mouse, and Rat samples, supporting translational work that compares endogenous NFU1 expression across rodent disease models and patient-derived human tissue. The polyclonal format maximizes coverage of epitopes across the NFU1 protein, which is helpful when probing patient samples that may carry missense variants affecting individual epitopes. Storage at 4 °C is appropriate for short-term use; for longer intervals, aliquoting and freezing at -20 °C preserves activity, and the glycerol component protects against freeze-thaw damage. The host species is Rabbit, clonality is polyclonal, and the conjugate is unconjugated, giving researchers flexibility to pair the antibody with HRP, fluorescent, or biotinylated secondary reagents of their choice. For laboratory research use only; not intended for diagnostic or therapeutic applications.