Anti-Dystonin (DST) Polyclonal Antibody for WB, ELISA - Q03001

Core Product Specifications and Parameters

Biological Background: Dystonin Function and Localization

• Dystonin (DST), also known as bullous pemphigoid antigen 1 (BPAG1) or 230/240 kDa bullous pemphigoid antigen, is a large cytoskeletal linker protein belonging to the plakin family. Multiple isoforms are generated through alternative promoter usage and splicing, with isoform 1 expressed in myoblasts, isoform 3 in skin, and isoform 6 in brain.
• Dystonin integrates intermediate filaments, actin, and microtubule cytoskeleton networks. It anchors keratin-containing intermediate filaments to hemidesmosomes in epithelial cells, playing a structural role in hemidesmosome assembly and regulation of keratinocyte polarity and motility via ITGB4-RAC1 signaling.
• In neural and muscle cells, DST is required for anchoring intermediate filaments to the actin cytoskeleton and for bundling actin filaments around the nucleus. It also regulates microtubule network organization and stability in sensory neurons to support axonal transport.
• Subcellular localization is highly diverse: dystonin is found at hemidesmosomes, the cytoplasm, stress fibers, cell cortex, axons, sarcomeric Z-lines and H-zones, nuclear envelope, and endoplasmic reticulum membrane, reflecting its multifaceted linker functions.
• Key post-translational modifications and structural features include calcium-binding, coiled-coil regions, SH3 domain, and a CH domain; it is a phosphoprotein, palmitoylated lipoprotein, and contains isopeptide bonds. It also has transmembrane regions and is involved in ubiquitin conjugation.
• Tissue-specific expression highlights its role in muscle, skin, and neuronal tissues: highly expressed in skeletal muscle and cultured keratinocytes, with distinct isoforms contributing to tissue-specific cytoskeletal organization and mechanical integrity.
• Mutations in DST are associated with epidermolysis bullosa simplex and hereditary sensory and autonomic neuropathy type 6, underscoring its critical role in maintaining tissue resilience.
• Additional functions include mediating docking of the dynein/dynactin motor complex to vesicle cargos for retrograde axonal transport via interactions with TMEM108 and DCTN1, linking cytoskeleton to intracellular trafficking.

Experimental Guidance and Technical Tips

• The immunogen is a recombinant fusion protein corresponding to the N-terminal 270 amino acids of human DST (NP_001714.1). For Western blot, consider that full-length dystonin is extremely large (~861 kDa), so ensure appropriate gel conditions (e.g., low-percentage acrylamide) and extended transfer times. Detection of smaller isoforms may also occur.
• For ELISA applications, validate coating conditions and antibody titration using recombinant protein or cell lysates from relevant species (human, mouse, rat). Due to its polyclonal nature, batch-to-batch consistency should be assessed if used for quantitative assays.
• Cross-reactivity with mouse and rat is specified; however, confirm endogenous expression in target tissues or cell lines, as dystonin isoforms vary. Endogenous levels may be low in some commonly used cell lines.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio provides diagnostic manufacturers and research laboratories with streamlined access to IVD raw materials, including validated antibodies for cytoskeletal and adhesion targets like dystonin. Beyond this polyclonal antibody, we offer one-stop sourcing for matched antibody pairs, optimized monoclonal alternatives, bulk ancillary reagents, and rare-target procurement to support your assay development from concept to clinic.