Rabbit anti-Human, Mouse USH1C Polyclonal Antibody | anti-USH1C antibody
USH1C, NT (USH1C, AIE75, 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)
Purified by Protein A affinity chromatography.
Purified by Protein A affinity chromatography.
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500
Western Blot (WB)
(USH1C Antibody (N-term) western blot analysis in K562 cell line lysates (35ug/lane).This demonstrates the USH1C antibody detected the USH1C protein (arrow).)
Western Blot (WB)
(USH1C Antibody (N-term) western blot analysis in mouse NIH-3T3 cell line lysates (35ug/lane).This demonstrates the USH1C antibody detected the USH1C protein (arrow).)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a scaffold protein that functions in the assembly of Usher protein complexes. The protein contains PDZ domains, a coiled-coil region with a bipartite nuclear localization signal and a PEST degradation sequence. Defects in this gene are the cause of Usher syndrome type 1C and non-syndromic sensorineural deafness autosomal recessive type 18. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009]
Uniprot Description
Function: Required for normal development and maintenance of cochlear hair cell bundles. 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 hearing
By similarity.
Subunit structure: Interacts with F-actin. Interacts with HARP
By similarity. Interacts with USH1G/SANS, USH2A and SLC4A7. Interacts (via the first PDZ domain) with the C-terminus of USHBP1. Interacts (via N-terminus and second PDZ domain) with CDH23. Part of a complex composed of USH1C, USH1G and MYO7A. Ref.9 Ref.11 Ref.13 Ref.14 Ref.16 Ref.17
Subcellular location: Cytoplasm › cytosol. Cytoplasm › cytoskeleton. Note: Colocalizes with F-actin
By similarity. Detected at the tip of cochlear hair cell stereocilia. Ref.14 Ref.16 Ref.17
Tissue specificity: Expressed in small intestine, colon, kidney, eye and weakly in pancreas. Expressed also in vestibule of the inner ear. Ref.11
Domain: The PDZ domain 1 mediates interactions with USH1G/SANS and SLC4A7. Ref.17The N-terminal region constitutes an independently folded domain that has structural similarity with the CCM2 C-terminus, despite very low sequence similarity. Ref.17
Involvement in disease: Usher syndrome 1C (USH1C) [MIM:276904]: USH is a genetically heterogeneous condition characterized by the association of retinitis pigmentosa with sensorineural deafness. Age at onset and differences in auditory and vestibular function distinguish Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2) and Usher syndrome type 3 (USH3). USH1 is characterized by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.8Deafness, autosomal recessive, 18A (DFNB18A) [MIM:602092]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.10
Sequence similarities: Contains 3 PDZ (DHR) domains.
Sequence caution: The sequence AAC18049.1 differs from that shown. Reason: Frameshift at position 552.
Research Articles on USH1C
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Product Notes
The USH1C ush1c (Catalog #AAA647772) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The USH1C, NT (USH1C, AIE75, 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) reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's USH1C can be used in a range of immunoassay formats including, but not limited to, ELISA (EL/EIA), Western Blot (WB). Suitable for use in Western Blot, ELISA Dilution: ELISA: 1:1,000 Western Blot: 1:100-500. Researchers should empirically determine the suitability of the USH1C ush1c for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "USH1C, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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