Aconitase 1 (ACO1), also known as iron regulatory protein 1 (IRP1), is a bifunctional mitochondrial enzyme involved in cellular iron homeostasis and energy metabolism. It catalyzes the isomerization of citrate to isocitrate in the tricarboxylic acid (TCA) cycle, relying on an iron-sulfur (4Fe-4S) cluster for enzymatic activity. Under low iron conditions, ACO1 loses this cluster and switches to its IRP1 form, binding to iron-responsive elements (IREs) in mRNAs to regulate iron uptake, storage, and utilization. Aconitase 1 antibodies are essential tools for studying its dual roles in metabolic regulation and iron sensing. These antibodies are widely used in techniques like Western blotting, immunohistochemistry (IHC), and immunofluorescence (IF) to detect ACO1 expression levels, subcellular localization, and tissue distribution. Researchers employ them to explore pathologies linked to ACO1 dysfunction, including neurodegenerative diseases (e.g., Alzheimer’s), cancer (where altered TCA cycle activity affects tumor growth), and iron-related disorders. Validated antibodies typically target conserved regions of ACO1 across species like humans, mice, and rats. Specificity is critical due to structural similarities with Aconitase 2 (ACO2), another isoform with distinct subcellular localization. Proper controls, such as knockout validation, are recommended to ensure accurate interpretation in experimental models.