ACOX1 (acyl-Coenzyme A oxidase 1) is the rate-limiting enzyme in peroxisomal fatty acid oxidation, catalyzing the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs . Defects in ACOX1 are linked to pseudoneonatal adrenoleukodystrophy (Pseudo-NALD), a rare neurodegenerative disorder . Antibodies against ACOX1 are essential tools for studying its expression, localization, and dysfunction in disease models.
ACOX1 deficiency: In vitro studies using patient-derived fibroblasts demonstrated elevated very-long-chain fatty acids (VLCFA), a hallmark of Pseudo-NALD. Niclosamide, identified via drug screening, reduced VLCFA levels by ~40% in patient cells .
Therapeutic insights: Long-term niclosamide administration in a pediatric patient showed a favorable safety profile, highlighting ACOX1’s role as a therapeutic target .
Subcellular localization: ACOX1 antibodies (e.g., ab184032 ) confirmed enzyme localization to peroxisomes via immunofluorescence.
Protein interactions: Co-immunoprecipitation assays revealed ACOX1’s association with metabolic regulators like PPARα .
Biomarker potential: ACOX1 expression correlates with renal fibrosis progression, as shown in murine models .
Diagnostic utility: Immunohistochemistry using HPA028759 detects ACOX1 in human tissue arrays, aiding in disease stratification.
Applications : Immunostainings
Sample type: cell
Review: Representative images of ACOX1, FASN, PPARγ, and SCD1 staining. The transcription levels of genes involved in lipid metabolism, such as Acaca, Cd36, Fasn, Fabp4, and Scd1, were dramatically increased in the liver of shAcox1-infected (shACOX1) NCD mouse.
Here’s a structured collection of FAQs tailored for researchers working with ACX1 antibodies, incorporating experimental design considerations, methodological insights, and data analysis principles:
ACX1 exhibits context-dependent roles:
Plant studies (Arabidopsis): ACX1/5 double mutants show:
Mammalian systems: ACX1 regulates peroxisomal fatty acid β-oxidation.
Methodological recommendation:
Recent advances combine:
Step 1: Compare epitope sequences across species (e.g., human vs. pig ACX1).
Step 2: Use recombinant protein spike-in assays.
Step 3: Perform peptide blocking experiments (pre-incubate antibody with immunogen) .