coq-8 Antibody

COQ-8 Antibody: Target and Functional Context

The COQ8 protein (also known as ADCK3) belongs to the ancient UbiB/ADCK family of atypical protein kinases. It plays an essential role in coenzyme Q (CoQ) biosynthesis, a redox-active lipid critical for mitochondrial electron transport and antioxidant defense. Key features include:

COQ-8 antibodies are used to study this protein’s interactions, subcellular localization, and role in diseases linked to CoQ deficiency (e.g., ataxia, mitochondrial disorders) .

Applications in Research

COQ-8 antibodies are employed in diverse experimental workflows:

Western Blotting (WB)

• Purpose: Quantify COQ8 levels in lysates or purified mitochondrial fractions.

• Key Findings:

  • Overexpression of COQ8 rescues CoQ biosynthesis defects in coq null mutants by stabilizing unstable Coq polypeptides (Coq4, Coq6, Coq7, Coq9) .

  • Suppression of COQ8 expression reduces CoQ levels and cellular respiration .

Immunohistochemistry (IHC)

• Purpose: Visualize COQ8 localization in tissue sections.

• Relevant Studies:

  • Mitochondrial targeting sequence and transmembrane domain deletion mutants show disrupted membrane association .

ELISA and Other Assays

• Purpose: Measure COQ8 protein levels in serum or cell lysates.

• Commercial Kits: Sandwich ELISA kits (e.g., ABIN6225943) enable quantitative analysis .

CoQ Deficiency and Ataxia

• Disease Link: Mutations in COQ8A cause recessive ataxia with CoQ10 deficiency .

• Mechanism: Disrupted CoQ biosynthesis leads to impaired mitochondrial function and oxidative stress .

Small Molecule Modulation

• Inhibitors: 4-anilinoquinolines (e.g., UNC-CA157) suppress COQ8A activity, reducing de novo CoQ synthesis .

• Activators: 2-propylphenol (2-PP) enhances COQ8 ATPase activity, potentially aiding CoQ supplementation strategies .

Membrane Binding Dynamics

• Lipid Interactions: Cardiolipin (CL) modulates COQ8 membrane association via conserved KxGQ domain residues .

Optimization and Validation

• WB Protocol: Dilute antibodies 1:500–1:2000; use mitochondrial-enriched lysates .

• IHC Protocol: Optimal dilution 1:50–1:200; fixative-dependent optimization required .

• Cross-Reactivity: Avoid using antibodies validated only in human samples for rodent models .