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BRAND / VENDOR: Abcam

Abcam, ab133763, Anti-USH1C/Harmonin antibody [EPR8131]

CATALOG NUMBER: ab133763
Regular price$0.99
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Product Description

Size: 100µL / 1mL
Rabbit Recombinant Monoclonal USH1C/Harmonin antibody. Suitable for WB and reacts with Human samples. Cited in 2 publications.
Key facts
Host species:Rabbit,
Clonality:Monoclonal,
Clone number:EPR8131,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:The exact immunogen used to generate this antibody is proprietary information.

Product details:
Patented technology
Our RabMAb
technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to
RabMAb® patents
What are the advantages of a recombinant monoclonal antibody?
This product is a recombinant monoclonal antibody, which offers several advantages including:
- High batch-to-batch consistency and reproducibility
- Improved sensitivity and specificity
- Long-term security of supply
- Animal-free batch production
For more information, read more on
recombinant antibodies

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.2 - 7.4Preservative: 0.01% Sodium azideConstituents: PBS, 50% Tissue culture supernatant, 40% Glycerol (glycerin, glycerine), 0.05% BSA, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Storage information-Stable for 12 months at -20°C

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.


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Collaboration

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