| Field | Specification |
|---|---|
| Mfr No | |
| Clonality | |
| Host | |
| Immunogen | E.coli-derived human COQ8B recombinant protein (Position: Q204-A527) was used as the immunogen for the COQ8B antibody. |
| Isotype | |
| Product Type | |
| Purity | |
| Reactivity | |
| Storage | |
| Target | |
| UniProt # |
Overview
COQ8B Antibody / Coenzyme Q8B is a anti-COQ8B Rabbit antibody Polyclonal (rabbit origin) supplied in Lyophilized format. Recommended for workflows such as Western blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF), ELISA with listed reactivity in Human, Mouse, Rat. Reported localization: Cytoplasm, Mitochondria, Cell membrane.
Key elements and design rationale
- Target: COQ8B
- Antibody details: Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG
- Format: Lyophilized
- Applications (as listed): WB, IHC, ICC, IF, ELISA
Biological background
COQ8B antibody identifies a protein essential for maintaining efficient oxidative phosphorylation and ATP production. Although it contains a kinase-like domain, COQ8B functions primarily as a regulatory or scaffolding enzyme rather than a classical kinase. It interacts with other coenzyme Q biosynthesis enzymes such as COQ6, COQ7, and COQ9 to ensure proper assembly and function of the multiprotein COQ complex. Loss of COQ8B activity leads to coenzyme Q deficiency, impaired electron transport, and reduced ATP generation.
Clinically, mutations in COQ8B are associated with primary coenzyme Q10 deficiency type 9 (COQ10D9), a mitochondrial disorder characterized by steroid-resistant nephrotic syndrome, progressive kidney failure, and neurological symptoms. Patients with COQ8B mutations show mitochondrial structural abnormalities and decreased oxidative capacity. Early diagnosis and coenzyme Q10 supplementation can partially restore mitochondrial function and improve clinical outcomes. The COQ8B gene is highly expressed in kidney, heart, and skeletal muscle, correlating with tissues that have high energy demands.
Structurally, COQ8B belongs to the UbiB family of atypical kinases that share sequence similarity with the AarF domain-containing proteins involved in lipid and isoprenoid metabolism. Its conserved nucleotide-binding pocket and regulatory loop regions support ATP binding, suggesting an evolutionary link between metabolic sensing and lipid biosynthesis regulation. In addition to coenzyme Q production, COQ8B may influence mitochondrial morphology and stress signaling pathways related to ROS detoxification.
Functional studies demonstrate that COQ8B deficiency alters mitochondrial membrane potential and triggers compensatory upregulation of antioxidant enzymes. The protein�s dual role in coenzyme Q biosynthesis and mitochondrial quality control underscores its importance for cellular energy metabolism and redox balance. In renal physiology, COQ8B maintains podocyte mitochondrial function, and loss of function leads to glomerular injury and nephrotic syndrome. This disease relevance makes COQ8B antibody a valuable tool for both mitochondrial research and renal pathology studies.
Immunohistochemical staining using COQ8B antibody shows mitochondrial localization in kidney, heart, and skeletal muscle tissues. COQ8B antibody from
Research relevance and current trends
- Connecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).
- Considering isoforms and post-translational regulation when interpreting protein-level changes.
- Comparing results across species and model systems with matched controls.
Common research applications
- Western blotting: compare relative abundance and activation-state changes across conditions.
- Immunofluorescence: visualize subcellular distribution and cell-to-cell heterogeneity.
- Immunohistochemistry: map target signal in tissue context and compare regions/phenotypes.
- ELISA: support antibody-based quantification in assay formats where applicable.
Interpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.
Notes for experimental interpretation
- Signal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.
- Species differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.
Antibody notes: Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.
Customization & Add-ons: Can’t find the antibody you need—or require a custom format for your assay? We can help you source the best match or support custom antibody solutions for diverse research needs, including species and isotype selection, conjugations and labeling (e.g., HRP/AP, biotin, fluorophores), purification grade options (Protein A/G, affinity purified), formulation preferences (buffer selection, carrier-free, glycerol-free), custom concentrations and aliquoting, low-endotoxin options for cell-based work, and application-focused QC/validation support (project dependent). Click Talk to a Scientist to submit a request, email us at support@biohippo.com, or explore our Research Services for additional support—our team will follow up with feasibility details and next steps.
