{"product_id":"nfu1-antibody-hira-interacting-protein-5-hirip5-bha17135334","title":"NFU1 Antibody \/ HIRA-interacting protein 5 \/ HIRIP5","description":"\u003ch2\u003eOverview\u003c\/h2\u003e\u003cp\u003eNFU1 Antibody \/ HIRA-interacting protein 5 \/ HIRIP5 is a anti-NFU1 Rabbit antibody Polyclonal (rabbit origin) supplied in Lyophilized format. Recommended for workflows such as Western blot (WB), Immunocytochemistry (ICC), Immunofluorescence (IF), Flow cytometry (FACS), ELISA with listed reactivity in Human, Mouse, Rat. Reported localization: Cytoplasm, Mitochondria.\u003c\/p\u003e\u003ch2\u003eKey elements and design rationale\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eTarget:\u003c\/strong\u003e NFU1\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eAntibody details:\u003c\/strong\u003e Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFormat:\u003c\/strong\u003e Lyophilized\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eApplications (as listed):\u003c\/strong\u003e WB, ICC\/IF, FACS, ELISA\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eBiological background\u003c\/h2\u003e\u003cdiv\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.\u003c\/div\u003e\u003ch2\u003eResearch relevance and current trends\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eConnecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).\u003c\/li\u003e\n\u003cli\u003eConsidering isoforms and post-translational regulation when interpreting protein-level changes.\u003c\/li\u003e\n\u003cli\u003eComparing results across species and model systems with matched controls.\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eCommon research applications\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eWestern blotting:\u003c\/strong\u003e compare relative abundance and activation-state changes across conditions.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eImmunofluorescence:\u003c\/strong\u003e visualize subcellular distribution and cell-to-cell heterogeneity.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFlow cytometry:\u003c\/strong\u003e quantify target-positive populations and signal shifts at single-cell resolution.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eELISA:\u003c\/strong\u003e support antibody-based quantification in assay formats where applicable.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003eInterpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.\u003c\/p\u003e\u003ch2\u003eNotes for experimental interpretation\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eSignal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.\u003c\/li\u003e\n\u003cli\u003eSpecies differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003e\u003cstrong\u003eAntibody notes:\u003c\/strong\u003e Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.\u003c\/p\u003e\u003c!-- Sources (internal): - UniProt search — UniProt — https:\/\/www.uniprot.org\/uniprotkb?query=NFU1 - NCBI Gene search — NCBI — https:\/\/www.ncbi.nlm.nih.gov\/gene\/?term=NFU1 - Ensembl search — Ensembl — https:\/\/www.ensembl.org\/Multi\/Search\/Results?q=NFU1 - Human Protein Atlas search — HPA — https:\/\/www.proteinatlas.org\/search\/NFU1 - PubMed (review) — NLM — https:\/\/pubmed.ncbi.nlm.nih.gov\/?term=NFU1+review --\u003e","brand":"NSJ Bioreagents","offers":[{"title":"Adding 0.2 ml of distilled water will yield a concentration of 500 ug\/ml \/ 100 ug","offer_id":53047286301037,"sku":"FY12431","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/get_image_052e1904-b795-46d1-8ad6-2d03e69bbaae.jpg?v=1782237037","url":"https:\/\/www.ebiohippo.com\/products\/nfu1-antibody-hira-interacting-protein-5-hirip5-bha17135334","provider":"BioHippo","version":"1.0","type":"link"}