Espin; Autosomal recessive deafness type 36 protein; Ectoplasmic specialization protein; ESPN; DFNB36

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This ESPN antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 17-45 amino acids from the N-terminal region of human ESPN.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-ESPN antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ESPN is a multifunctional actin-bundling protein. It plays a major role in regulating the organization, dimensions, dynamics, and signaling capacities of the actin filament-rich, microvillus-type specializations that mediate sensory transduction in various mechanosensory and chemosensory cells.

Neuroscience; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS)

WB~~1:1000

Western blot analysis of ESPN Antibody (N-term) in 293 cell line lysates (35ug/lane). ESPN (arrow) was detected using the purified Pab.

Formalin-fixed and paraffin-embedded human testis tissue reacted with ESPN Antibody (N-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.

ESPN Antibody (N-term) flow cytometric analysis of 293 cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

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espin

espin

Espin

DFNB36??[Similar Products]

ESPN_HUMAN

This gene encodes a multifunctional actin-bundling protein. It plays a major role in regulating the organization, dimensions, dynamics, and signaling capacities of the actin filament-rich, microvillus-type specializations that mediate sensory transduction in various mechanosensory and chemosensory cells. Mutations in this gene are associated with autosomal recessive neurosensory deafness, and autosomal dominant sensorineural deafness without vestibular involvement. [provided by RefSeq, Nov 2009]

ESPN: Multifunctional actin-bundling protein. Plays a major role in regulating the organization, dimensions, dynamics and signaling capacities of the actin filament-rich, microvillus-type specializations that mediate sensory transduction in variouS mechanosensory and chemosensory cells. Defects in ESPN are the cause of deafness autosomal recessive type 36 with or without vestibular involvement (DFNB36). A form of non-syndromic sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Actin-binding; Cytoskeletal

Chromosomal Location of Human Ortholog: 1p36.31

Cellular Component: filamentous actin; stereocilium; cytoplasm; stereocilium bundle tip; brush border

Molecular Function: actin filament binding; SH3 domain binding

Biological Process: positive regulation of filopodium formation; parallel actin filament bundle formation; sensory perception of sound; locomotory behavior; negative regulation of cytoskeleton organization and biogenesis

Disease: Deafness, Autosomal Recessive 36, With Or Without Vestibular Involvement

Boulouiz,R., et.al., Am. J. Med. Genet. A 146A (23), 3086-3089 (2008)

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