{"product_id":"coq8b-antibody-coenzyme-q8b-bha17136215","title":"COQ8B Antibody \/ Coenzyme Q8B","description":"\u003ch2\u003eOverview\u003c\/h2\u003e\u003cp\u003eCOQ8B 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.\u003c\/p\u003e\u003ch2\u003eKey elements and design rationale\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eTarget:\u003c\/strong\u003e COQ8B\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eAntibody details:\u003c\/strong\u003e Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFormat:\u003c\/strong\u003e Lyophilized\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eApplications (as listed):\u003c\/strong\u003e WB, IHC, ICC, IF, ELISA\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eBiological background\u003c\/h2\u003e\u003cdiv\u003eCOQ8B antibody detects Coenzyme Q8B, a mitochondrial inner membrane protein encoded by the COQ8B gene on chromosome 19q13.2. COQ8B, also known as ADCK4 (AarF domain-containing kinase 4), belongs to the atypical protein kinase-like (PKL) family and plays a vital role in the biosynthesis of coenzyme Q (ubiquinone), a key component of the mitochondrial respiratory chain. The COQ8B protein localizes to mitochondria, where it stabilizes the coenzyme Q biosynthetic complex and regulates redox homeostasis. Structurally, COQ8B possesses a kinase-like fold with an ATP-binding domain and transmembrane segment that anchors it to the inner mitochondrial membrane.\u003cbr\u003e\u003cbr\u003eCOQ8B 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.\u003cbr\u003e\u003cbr\u003eClinically, 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.\u003cbr\u003e\u003cbr\u003eStructurally, 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.\u003cbr\u003e\u003cbr\u003eFunctional 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.\u003cbr\u003e\u003cbr\u003eImmunohistochemical staining using COQ8B antibody shows mitochondrial localization in kidney, heart, and skeletal muscle tissues. COQ8B antibody from\u003c\/div\u003e\u003ch2\u003eResearch relevance and current trends\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eConnecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).\u003c\/li\u003e\n\u003cli\u003eConsidering isoforms and post-translational regulation when interpreting protein-level changes.\u003c\/li\u003e\n\u003cli\u003eComparing results across species and model systems with matched controls.\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eCommon research applications\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eWestern blotting:\u003c\/strong\u003e compare relative abundance and activation-state changes across conditions.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eImmunofluorescence:\u003c\/strong\u003e visualize subcellular distribution and cell-to-cell heterogeneity.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eImmunohistochemistry:\u003c\/strong\u003e map target signal in tissue context and compare regions\/phenotypes.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eELISA:\u003c\/strong\u003e support antibody-based quantification in assay formats where applicable.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003eInterpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.\u003c\/p\u003e\u003ch2\u003eNotes for experimental interpretation\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eSignal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.\u003c\/li\u003e\n\u003cli\u003eSpecies differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003e\u003cstrong\u003eAntibody notes:\u003c\/strong\u003e Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.\u003c\/p\u003e\u003c!-- Sources (internal): - UniProt search — UniProt — https:\/\/www.uniprot.org\/uniprotkb?query=COQ8B - NCBI Gene search — NCBI — https:\/\/www.ncbi.nlm.nih.gov\/gene\/?term=COQ8B - Ensembl search — Ensembl — https:\/\/www.ensembl.org\/Multi\/Search\/Results?q=COQ8B - Human Protein Atlas search — HPA — https:\/\/www.proteinatlas.org\/search\/COQ8B - PubMed (review) — NLM — https:\/\/pubmed.ncbi.nlm.nih.gov\/?term=COQ8B+review --\u003e","brand":"NSJ Bioreagents","offers":[{"title":"Adding 0.2 ml of distilled water will yield a concentration of 500 ug\/ml \/ 100 ug","offer_id":53047322181997,"sku":"FY13313","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/get_image_3a5beac3-54e8-4a08-b3db-eadb0b0fb87b.jpg?v=1782237107","url":"https:\/\/www.ebiohippo.com\/products\/coq8b-antibody-coenzyme-q8b-bha17136215","provider":"BioHippo","version":"1.0","type":"link"}