{"product_id":"nsj-fy12431-100ug","title":"NFU1 Antibody \/ HIRA-interacting protein 5 \/ HIRIP5, 100 ug","description":"\u003cp\u003eThe NFU1 antibody targets Iron-sulfur cluster scaffold protein NFU1, a mitochondrial protein encoded by the NFU1 gene that functions in iron-sulfur (Fe-S) cluster assembly. Iron-sulfur cluster scaffold protein NFU1 is required for the maturation of a subset of mitochondrial Fe-S enzymes involved in oxidative phosphorylation, lipoic acid synthesis, and redox regulation. The NFU1 antibody provides researchers with a vital tool for studying mitochondrial metabolism, protein biogenesis, and disorders related to Fe-S cluster deficiency.\u003cbr\u003e\u003cbr\u003eIron-sulfur cluster scaffold protein NFU1 acts downstream of the core Fe-S assembly machinery (ISC system) and transfers preassembled clusters to specific target proteins, including lipoic acid synthetase and succinate dehydrogenase. It contains a conserved C-terminal cysteine-rich domain that binds transient Fe-S clusters before delivery to recipient apoproteins. The NFU1 antibody enables localization and expression analysis, showing strong mitochondrial matrix staining consistent with its chaperone-like function in cluster transfer.\u003cbr\u003e\u003cbr\u003eMutations in the NFU1 gene cause Multiple Mitochondrial Dysfunction Syndrome 1 (MMDS1), a severe autosomal recessive disorder characterized by infantile encephalopathy, lactic acidosis, and respiratory-chain deficiency. The NFU1 antibody supports investigations into the molecular mechanisms of MMDS1 by allowing detection of protein loss or misfolding in patient cells. Loss of functional NFU1 impairs Fe-S enzyme assembly, reducing activity of complexes II and lipoic acid�dependent dehydrogenases, ultimately compromising cellular energy metabolism.\u003cbr\u003e\u003cbr\u003eBeyond Fe-S cluster assembly, Iron-sulfur cluster scaffold protein NFU1 contributes to the maintenance of redox balance and the regulation of reactive oxygen species. The NFU1 antibody supports quantitative studies in models of oxidative stress, aging, and mitochondrial dysfunction. Reduced NFU1 expression has been associated with altered mitochondrial respiration and metabolic imbalance in cancer and neurodegeneration.\u003cbr\u003e\u003cbr\u003eThe NFU1 antibody performs effectively in western blotting, immunofluorescence, and immunohistochemistry, producing strong mitochondrial staining. NSJ Bioreagents provides this antibody with validated specificity and reproducibility for use in mitochondrial biology, metabolic, and biochemical research. By enabling accurate detection of Iron-sulfur cluster scaffold protein NFU1, the NFU1 antibody supports studies into mitochondrial Fe-S cluster assembly, redox regulation, and the molecular basis of metabolic disorders.\u003c\/p\u003e","brand":"NSJ Bioreagents","offers":[{"title":"100 ug","offer_id":51552389366040,"sku":"FY12431","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/get_image_b7bed50e-c40d-4a8f-9810-ff6e8d712196.jpg?v=1768354121","url":"https:\/\/danabiosci.com\/products\/nsj-fy12431-100ug","provider":"Dana Bioscience","version":"1.0","type":"link"}