Abcam, ab244404, Anti-USH1C/Harmonin antibody

Size: 100µL
Rabbit Polyclonal USH1C/Harmonin antibody. Suitable for IHC-P, WB, ICC/IF and reacts with Human, Mouse samples. Immunogen corresponding to Recombinant Fragment Protein within Human USH1C aa 350-450.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Mouse, Human,
Applications:WB, ICC/IF, IHC-PSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human USH1C aa 350-450. The exact immunogen used to generate this antibody is proprietary information.Q9Y6N9

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% 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.
The USH1C also known as Harmonin or Harmoneem is a protein with a molecular weight of approximately 73 kDa. It plays a mechanical role in connecting the mechanosensory hair bundle of the inner ear and the junctional complex of the retina. Harmonin protein expression mainly occurs in sensory hair cells of the inner ear and photoreceptor cells of the retina. These areas show its critical involvement in maintaining function in the sensory systems.
Biological function summary
The Harmonin protein participates in establishing and maintaining the structure and function of the hair cells and photoreceptors. It is a part of the multi-protein complex known as the Usher protein complex which includes other proteins such as cadherin and myosin. This complex ensures proper cell signaling and structural support necessary for sensory perception related to hearing and vision.
Pathways
The Harmonin protein is involved in the mechanotransduction pathway and the visual phototransduction pathway. It plays a role in converting mechanical signals into electrical ones within hair cells and is similarly vital in photoreceptor cells. Through these pathways Harmonin interacts with proteins like protocadherin and myosin VIIa highlighting its function in signal transduction processes within sensory cells.
Harmonin mutations link to Usher syndrome type 1C a disorder characterized by sensorineural hearing loss and retinitis pigmentosa. Defective Harmonin interactions disturb the Usher protein complex leading to the symptoms of this syndrome. Proteins such as myosin VIIa also relate to Usher syndrome as they function alongside Harmonin in maintaining sensory cell integrity. Understanding these connections offers insights into disease mechanisms and potential therapeutic targets.