Anti-Harmonin (Ush1C)

Harmonin (UniProt: Q9ES64; also known as PDZ domain-containing protein, Usher syndrome type-1C protein homolog) is encoded by the Ush1c gene (Gene ID 72088) in murine species. Harmonin is an anchoring/scaffolding protein that is a part of the functional network formed by USH1C, USH1G, CDH23 and MYO7A that mediates mechanotransduction in cochlear hair cells. It is required for normal development and maintenance of cochlear hair cell bundles. Harmonin is reported to colocalize with F-actin and is detected at the tip of cochlear hair cell stereocilia. It is also enriched in microvilli of the intestinal brush border where as part of the intermicrovillar adhesion complex it plays a role in brush border differentiation, controlling microvilli organization and length. Harmonin contains PDZ domains, a coiled-coil region with a bipartite nuclear localization signal and a PEST degradation sequence. Defects in this gene are known to cause Usher syndrome type 1C and non-syndromic sensorineural deafness. Usher syndrome is characterized by profound hearing loss, vestibular dysfunction, and visual impairment.

~62 kDa observed. 68.19/96.50/102.3 kDa (mouse isoform a1/b2/b3; UniProt Q9ES64) calculated. Uncharacterized bands may be observed in some lysate(s).

His-tagged recombinant mouse harmonin (Ush1C) fragment corresponding to the region N-terminal to the second PDZ domain.

Anti-Harmonin (Ush1C), Cat. No. ABN1696, is a highly specific rabbit polyclonal antibody that targets Harmonin and has been tested in Immunofluorescence and Western Blotting.

Immunofluorescence Analysis: A 1-2 µg/mL from a representative lot immunostained connectting cilium of photoreceptor cells in frozen mouse retina tissue postfixed with paraformaldehyde (Courtesy of Dr. Tiansen Li at NIH).

Western Blotting Analysis: A 1-2 µg/mL from a representative lot detected harmonin (Ush1C) in mouse retina tissue lysate (Courtesy of Dr. Tiansen Li at NIH).

Research Category
Neuroscience

This rabbit polyclonal antibody targets the region N-terminal to the second PDZ domain present in all three murine harmonin (Ush1C) spliced isoforms reported by UniProt (Q9ES64).

Affinity purified.

Purified rabbit polyclonal antibody in PBS without azide with 50% glycerol.

Stable for 1 year at -20°C from date of receipt.
Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Evaluated by Western Blotting in mouse kidney tissue lysate.

Western Blotting Analysis: 2 µg/mL of this antibody detected harmonin (Ush1C) in 10 µg of mouse kidney tissue lysate.

Concentration: Please refer to lot specific datasheet.

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