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Mouse Anti-IMPG2 Recombinant Antibody (3H5) (CBMAB-A4456-LY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
3H5
Antibody Isotype
IgG2a, κ
Application
WB, ELISA

Basic Information

Immunogen
IMPG2 (NP_057331.2, 572 a.a. ~ 678 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human
Antibody Isotype
IgG2a, κ
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
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
interphotoreceptor matrix proteoglycan 2
Introduction
Interphotoreceptor matrix proteoglycan-2 is part of an extracellular complex occupying the interface between photoreceptors and the retinal pigment epithelium in the fundus of the eye.[supplied by OMIM
Entrez Gene ID
50939
UniProt ID
Q9BZV3
Alternative Names
IPM200; SPACRCAN
Function
Chondroitin sulfate- and hyaluronan-binding proteoglycan involved in the organization of interphotoreceptor matrix; may participate in the maturation and maintenance of the light-sensitive photoreceptor outer segment. Binds heparin.
Biological Process
Extracellular matrix organizationIEA:Ensembl
Protein localizationIEA:Ensembl
Retina morphogenesis in camera-type eyeIEA:Ensembl
Visual perceptionManual Assertion Based On ExperimentTAS:UniProtKB
Cellular Location
Photoreceptor outer segment membrane; Photoreceptor inner segment membrane; Secreted, extracellular space, extracellular matrix, interphotoreceptor matrix
Involvement in disease
Retinitis pigmentosa 56 (RP56):
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.
Macular dystrophy, vitelliform, 5 (VMD5):
A form of macular dystrophy, a retinal disease in which various forms of deposits, pigmentary changes, and atrophic lesions are observed in the macula lutea. Vitelliform macular dystrophies are characterized by yellow, lipofuscin-containing deposits, usually localized at the center of the macula. VMD5 features include late-onset moderate visual impairment and preservation of retinal pigment epithelium reflectivity.
Topology
Extracellular: 23-1099
Helical: 1100-1120
Cytoplasmic: 1121-1241
PTM
Highly glycosylated (N- and O-linked carbohydrates).
More Infomation

Castellini, M. E., Spagnolli, G., Poggi, L., Biasini, E., Casarosa, S., & Messina, A. (2023). Identification of the zebrafish homologues of IMPG2, a retinal proteoglycan. Cell and Tissue Research, 1-13.

Mayerl, S. J., Bajgai, S., Ludwig, A. L., Jager, L. D., Williams, B. N., Bacig, C., ... & Gamm, D. M. (2022). Human retinal organoids harboring IMPG2 mutations exhibit a photoreceptor outer segment phenotype that models advanced retinitis pigmentosa. Stem Cell Reports, 17(11), 2409-2420.

Vázquez-Domínguez, I., Li, C. H., Fadaie, Z., Haer-Wigman, L., Cremers, F. P., Garanto, A., ... & Roosing, S. (2022). Identification of a complex allele in IMPG2 as a cause of adult-onset vitelliform macular dystrophy. Investigative Ophthalmology & Visual Science, 63(5), 27-27.

Mitchell, B., Coulter, C., Geldenhuys, W. J., Rhodes, S., & Salido, E. M. (2022). Interphotoreceptor matrix proteoglycans IMPG1 and IMPG2 proteolyze in the SEA domain and reveal localization mutual dependency. Scientific Reports, 12(1), 15535.

Chatterjee, S., Gupta, S., Chaudhry, V. N., Chaudhry, P., Mukherjee, A., & Mutsuddi, M. (2022). Whole exome sequencing identifies a novel splice-site mutation in IMPG2 gene causing Stargardt-like juvenile macular dystrophy in a north Indian family. Gene, 816, 146158.

Shah, S. M., Schimmenti, L. A., Marmorstein, A. D., & Bakri, S. J. (2021). Adult-onset vitelliform macular dystrophy secondary to a novel IMPG2 gene variant. Retinal Cases and Brief Reports, 15(4), 356-358.

Hitti-Malin, R. J., Burmeister, L. M., Ricketts, S. L., Lewis, T. W., Pettitt, L., Boursnell, M., ... & Mellersh, C. S. (2020). A LINE-1 insertion situated in the promoter of IMPG2 is associated with autosomal recessive progressive retinal atrophy in Lhasa Apso dogs. BMC genetics, 21(1), 1-17.

Salido, E. M., & Ramamurthy, V. (2020). Proteoglycan IMPG2 shapes the interphotoreceptor matrix and modulates vision. Journal of Neuroscience, 40(20), 4059-4072.

Xu, H., Qu, C., Gan, L., Sun, K., Tan, J., Liu, X., ... & Zhang, L. (2020). Deletion of the Impg2 gene causes the degeneration of rod and cone cells in mice. Human Molecular Genetics, 29(10), 1624-1634.

Khan, A. O., & Al Teneiji, A. M. (2019). Homozygous and heterozygous retinal phenotypes in families harbouring IMPG2 mutations. Ophthalmic genetics, 40(3), 247-251.

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For research use only. Not intended for any clinical use.

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