Sign in or Register   Sign in or Register
  |  

Mouse Anti-USH2A Recombinant Antibody (CBXU-038) (CBMAB-U0154-CQ)

This product is a mouse antibody that recognizes USH2A. The antibody CBXU-038 can be used for immunoassay techniques such as: WB, IP, IF, ELISA.
See all USH2A antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXU-038
Antibody Isotype
IgG
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
usherin
Introduction
This gene encodes a protein that contains laminin EGF motifs, a pentaxin domain, and many fibronectin type III motifs. The protein is found in the basement membrane, and may be important in development and homeostasis of the inner ear and retina. Diseases associated with USH2A include Usher Syndrome, Type Iia and Retinitis Pigmentosa 39. Gene Ontology (GO) annotations related to this gene include collagen binding and myosin binding.
Entrez Gene ID
7399
UniProt ID
O75445
Alternative Names
US2; RP39; USH2; dJ1111A8.1
Function
Involved in hearing and vision as member of the USH2 complex. In the inner ear, required for the maintenance of the hair bundle ankle formation, which connects growing stereocilia in developing cochlear hair cells. In retina photoreceptors, the USH2 complex is required for the maintenance of periciliary membrane complex that seems to play a role in regulating intracellular protein transport.
Biological Process
Biological Process animal organ morphogenesis Source:GO_Central1 Publication
Biological Process cell migration Source:GO_Central1 Publication
Biological Process establishment of localization in cell Source:Ensembl
Biological Process establishment of protein localization Source:UniProtKB
Biological Process hair cell differentiation Source:BHF-UCL
Biological Process inner ear auditory receptor cell differentiation Source:Ensembl
Biological Process inner ear receptor cell differentiation Source:BHF-UCL
Biological Process maintenance of animal organ identity Source:HGNC-UCL1 Publication
Biological Process photoreceptor cell maintenance Source:HGNC-UCL1 Publication
Biological Process response to stimulus Source:UniProtKB-KW
Biological Process sensory perception of light stimulus Source:HGNC-UCL1 Publication
Biological Process sensory perception of sound Source:HGNC-UCL1 Publication
Biological Process substrate adhesion-dependent cell spreading Source:GO_Central1 Publication
Biological Process tissue development Source:GO_Central1 Publication
Biological Process visual perception Source:UniProtKB-KW
Cellular Location
Cell projection, stereocilium membrane
Component of the interstereocilia ankle links in the inner ear sensory cells. In photoreceptors, localizes at a plasma membrane microdomain in the apical inner segment that surrounds the connecting cilia called periciliary membrane complex.
Isoform 2
Secreted
Involvement in disease
Usher syndrome 2A (USH2A):
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). USH2 is characterized by congenital mild hearing impairment with normal vestibular responses.
Retinitis pigmentosa 39 (RP39):
A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.
Topology
Extracellular: 32-5042
Helical: 5043-5063
Cytoplasmic: 5064-5202
Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-USH2A Recombinant Antibody (CBXU-038)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare