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AB239541

Recombinant Human USH1C/Harmonin protein (Tagged)

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Recombinant Human USH1C/Harmonin protein (Tagged) is a Human Full Length protein, in the 1 to 533 aa range, expressed in Escherichia coli, with >90%, suitable for SDS-PAGE.

View Alternative Names

AIE75, USH1C, Harmonin, Antigen NY-CO-38/NY-CO-37, Autoimmune enteropathy-related antigen AIE-75, Protein PDZ-73, Renal carcinoma antigen NY-REN-3, Usher syndrome type-1C protein

1 Images
SDS-PAGE - Recombinant Human USH1C/Harmonin protein (Tagged) (AB239541)
  • SDS-PAGE

Supplier Data

SDS-PAGE - Recombinant Human USH1C/Harmonin protein (Tagged) (AB239541)

(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) analysis with 5% enrichment gel and 15% separation gel of ab239541.

Key facts

Purity

>90% SDS-PAGE

Expression system

Escherichia coli

Tags

His tag N-Terminus

Applications

SDS-PAGE

applications

Biologically active

No

Accession

Q9Y6N9

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 7.2 - 7.4 Constituents: Tris buffer, 50% Glycerol (glycerin, glycerine)

storage-buffer

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Product details

This product was previously labelled as USH1C
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Sequence info

[{"sequence":"MDRKVAREFRHKVDFLIENDAEKDYLYDVLRMYHQTMDVAVLVGDLKLVINEPSRLPLFDAIRPLIPLKHQVEYDQLTPRRSRKLKEVRLDRLHPEGLGLSVRGGLEFGCGLFISHLIKGGQADSVGLQVGDEIVRINGYSISSCTHEEVINLIRTKKTVSIKVRHIGLIPVKSSPDEPLTWQYVDQFVSESGGVRGSLGSPGNRENKEKKVFISLVGSRGLGCSISSGPIQKPGIFISHVKPGSLSAEVGLEIGDQIVEVNGVDFSNLDHKEGRELFMTDRERLAEARQRELQRQELLMQKRLAMESNKILQEQQEMERQRRKEIAQKAAEENERYRKEMEQIVEEEEKFKKQWEEDWGSKEQLLLPKTITAEVHPVPLRKPKYDQGVEPELEPADDLDGGTEEQGEQDFRKYEEGFDPYSMFTPEQIMGKDVRLLRIKKEGSLDLALEGGVDSPIGKVVVSAVYERGAAERHGGIVKGDEIMAINGKIVTDYTLAEAEAALQKAWNQGGDWIDLVVAVCPPKEYDDELTFF","proteinLength":"Full Length","predictedMolecularWeight":"76.3 kDa","actualMolecularWeight":null,"aminoAcidEnd":533,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Escherichia coli","accessionNumber":"Q9Y6N9","tags":[{"tag":"His","terminus":"N-Terminus"}]}]
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Properties and storage information

Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C
Storage information
Avoid freeze / thaw cycle
False
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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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Specifications

Form

Liquid

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General info

Function

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. Required for normal development and maintenance of cochlear hair cell bundles (By similarity). As part of the intermicrovillar adhesion complex/IMAC plays a role in brush border differentiation, controlling microvilli organization and length. Probably plays a central regulatory role in the assembly of the complex, recruiting CDHR2, CDHR5 and MYO7B to the microvilli tips (PubMed : 24725409, PubMed : 26812018).

Subcellular localisation

Cytoskeleton

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Product protocols

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Target data

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. Required for normal development and maintenance of cochlear hair cell bundles (By similarity). As part of the intermicrovillar adhesion complex/IMAC plays a role in brush border differentiation, controlling microvilli organization and length. Probably plays a central regulatory role in the assembly of the complex, recruiting CDHR2, CDHR5 and MYO7B to the microvilli tips (PubMed : 24725409, PubMed : 26812018).
See full target information USH1C
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