Abcam, ab234644, Anti-Dystonin/BPA antibody

Size: 100µg / 100µL
Rabbit Polyclonal Dystonin/BPA antibody. Suitable for WB, ICC/IF and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human DST aa 1-200.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:ICC/IF, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human DST aa 1-200. The exact immunogen used to generate this antibody is proprietary information.Q03001

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein G, Purification notes-Purity >95%., Storage buffer-pH: 7.4Preservative: 0.03% Proclin 300Constituents: PBS, 50% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Dystonin also known as BPA (Bullous Pemphigoid Antigen) is a large protein with a molecular mass of approximately 800 kDa. This versatile protein plays a mechanical role by providing structural integrity to cells particularly within tissues that experience mechanical stress. Dystonin locates mainly in the cytoskeleton-associated regions of muscle and neural cells where it connects cytoskeletal components to membrane complexes. The presence of dystonin in these areas indicates its function in maintaining tissue cohesion and stability under stress.
Biological function summary
Dystonin acts as a cytoskeletal linker that maintains cellular architecture by directly linking intermediate filaments to the actin and microtubule networks. As part of this structural network dystonin forms complexes with other proteins to stabilize cell junctions and muscle fibers. In neural tissues it plays an important role in maintaining the integrity of axons essential for proper nerve function. Dystonin ensures cells retain their shape and positioning which is necessary for regular cellular functions and signal transmissions in these tissues.
Pathways
Dystonin interacts with multiple structural proteins in the cytoskeletal pathway such as keratins and microtubule-associated proteins. The linkage function of dystonin integrates it into the hemidesmosomal pathway which is vital for epithelial cell adhesion. Dystonin works along with other proteins like integrins and plectin which also engage in these pathways promoting cellular adhesion and resilience against mechanical stress.
Mutations or deficiencies in dystonin link to diseases like bullous pemphigoid and Hereditary Sensory and Autonomic Neuropathy Type VI (HSAN VI). Bullous pemphigoid is an autoimmune disorder characterized by blistering of the skin due to compromised cell adhesion. The lack of functional dystonin in HSAN VI leads to nerve degradation highlighting its role in neural maintenance. Proteins like integrins are also implicated in these conditions emphasizing the importance of dystonin-related pathways in maintaining cell adhesion and nerve integrity.