COX10 Antibody

Research Applications and Findings

COX10 antibodies are pivotal in studying mitochondrial disorders, immune cell metabolism, and oxidative phosphorylation (OXPHOS):

Mitochondrial Myopathy and COX Deficiency

• Muscle-Specific COX10 Knockout (KO) Mice:

  • Exhibited severe COX deficiency, progressive myopathy, and mitochondrial proliferation (ragged-red fibers) .

  • Impaired Complex IV assembly confirmed via BN-PAGE and activity assays .

  • Key Data:

    • COX activity reduced by >90% in KO skeletal muscle .

    • Citrate synthase activity increased, indicating mitochondrial proliferation .

Antiviral Immunity and NK Cell Metabolism

• NK-Specific COX10 Deletion (Ncr1-Cox10 Δ/Δ Mice):

  • Impaired expansion of Ly49H+ NK cells during murine cytomegalovirus (MCMV) infection .

  • Metabolic Shift: COX10-deficient NK cells upregulated glycolysis but failed to sustain antiviral responses .

  • Key Data:

    • Spleen Ly49H+ NK cells reduced by ~50% at infection peak .

    • Oxygen consumption rate (OCR) decreased, while glycolysis (ECAR) increased .

T Cell Activation and Endothelial Metabolism

• T Cells: COX10 is critical for T cell quiescence exit and activation .

• Endothelial Cells: COX10 deletion prevents metabolic switch to OXPHOS, impairing angiogenesis .

Clinical Relevance of COX10 Dysfunction

Mutations in COX10 are linked to mitochondrial disorders, including:

• Leigh Syndrome: Neurodegeneration, lactic acidosis, and COX deficiency .

• Cardiomyopathy: Fatal infantile hypertrophic cardiomyopathy .

• Tubulopathy and Leukodystrophy: Renal and white matter abnormalities .

Diagnostic Use: COX10 antibodies aid in detecting protein levels in patient fibroblasts or tissue biopsies to confirm COX assembly defects .

Technical Considerations for COX10 Antibodies

• Sample Preparation: Use mitochondrial-enriched fractions for WB .

• Controls: Include positive controls like fetal lung lysate (Avantor) or HEK293T cells overexpressing COX10 .

• Limitations:

  • Not validated for immunohistochemistry in all species .

  • Cross-reactivity with rodent COX10 varies by vendor .

Emerging Insights from Recent Studies

• COA8 Interaction: COA8 stabilizes COX10 during oxidative stress, enhancing heme A synthesis .

• Therapeutic Strategies: Gene therapy and small-molecule chaperones are explored to rescue COX10 mutations .