NFU1 antibody (AA 42-254)

Catalog No. ABIN7874919

The Rabbit Polyclonal anti-NFU1 antibody is suitable to detect NFU1 in samples from Human, Mouse and Rat. It has been validated for WB, ELISA, IF, FACS and ICC.

Quick Overview for NFU1 antibody (AA 42-254) (ABIN7874919)

Target: NFU1 (NFU1 Iron-Sulfur Cluster Scaffold (NFU1))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This NFU1 antibody is un-conjugated

Application: Western Blotting (WB), ELISA, Immunofluorescence (IF), Flow Cytometry (FACS), Immunocytochemistry (ICC)

Product Details anti-NFU1 Antibody

Binding Specificity: AA 42-254

Purpose: NFU1 Antibody / HIRA-interacting protein 5 / HIRIP5

Purification: Immunogen affinity purified

Immunogen: E.coli-derived human NFU1 recombinant protein (Position: Q42-P254) was used as the immunogen for the NFU1 antibody.

Isotype: IgG

Application Details

Application Notes: Optimal dilution of the NFU1 antibody should be determined by the researcher.

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Storage: 4 °C,-20 °C

Storage Comment: After reconstitution, the NFU1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Target Details for NFU1

Target: NFU1 (NFU1 Iron-Sulfur Cluster Scaffold (NFU1))

Alternative Name: NFU1

Background: The 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.

Iron-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.

Mutations 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 acidi 1/2dependent dehydrogenases, ultimately compromising cellular energy metabolism.

Beyond 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.

The 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.

UniProt: Q9UMS0