X-linked retinitis pigmentosa GTPase regulator, ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

This assay has high sensitivity and excellent specificity for detection of Mouse RPGR. No significant cross-reactivity or interference between Mouse RPGR and analogues was observed.

Store at 2-8 degree C.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of RPGR elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS283129 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the X-linked retinitis pigmentosa GTPase regulator (RPGR) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing RPGR. The ELISA analytical biochemical technique of the MBS283129 kit is based on RPGR antibody-RPGR antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect RPGR antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, RPGR. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate RPGR in samples. An antibody specific for RPGR has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyRPGR present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for RPGR is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of RPGR bound in the initial step. The color development is stopped and the intensity of the color is measured.

146,029 Da

retinitis pigmentosa GTPase regulator

X-linked retinitis pigmentosa GTPase regulator

X-linked retinitis pigmentosa GTPase regulator

mRpgr  [Similar Products]

RPGR: a guanine nucleotide exchange factor (GEF) that plays a role in cilia biogenesis and maintenance. Interacts with the GDP-bound form of the small GTPase RAB8A. Promotes the exchange of GDP to GTP, converting inactive GDP-bound Rab proteins into their active GTP-bound form. Probably regulates cilia formation by regulating actin stress filaments and cell contractility. Plays an important role in photoreceptor integrity. May play a critical role in spermatogenesis and in intraflagellar transport processes May be involved in microtubule organization and regulation of transport in primary cilia. Interacts with PDE6D, RPGRIP1, CEP290, and DFNB31. Defects in RPGR are the cause of retinitis pigmentosa (RP3, RP15, and RPDSI), cone-rod dystrophy, X-linked 1 (CORDX1), and macular degeneration, X-linked, atrophic (MDXLA). Six isoforms of the human protein are produced by alternative splicing. Isoform 3 is found only in the retina. Colocalizes with RPGRIP1 in the outer segment of rod photoreceptors and cone outer segments. Isoform 6 associates with microtubule organizing centers, cilium basal bodies, and cilium axonemes. Interacts with NPM1 (via C-terminus), RPGRIP1L, SMC1A and SMC3.

Protein type: GEF; GEF, Rab

Chromosomal Location of Human Ortholog: X|X A1.1

Cellular Component: cell projection; centrosome; cilium; cytoplasm; cytoskeleton; Golgi apparatus; photoreceptor connecting cilium; photoreceptor outer segment

Molecular Function: guanyl-nucleotide exchange factor activity; protein binding; RNA binding

Biological Process: cell projection organization; eye photoreceptor cell development; intraflagellar transport; response to stimulus; retina morphogenesis in camera-type eye; visual perception

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