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

This assay has high sensitivity and excellent specificity for detection of Human RP2. No significant cross-reactivity or interference between Human RP2 and analogues was observed.

Store at 2-8 degree C.

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Small volumes of RP2 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.

MBS281436 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Protein XRP2, 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 RP2. The ELISA analytical biochemical technique of the MBS281436 kit is based on RP2 antibody-RP2 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect RP2 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, RP2. 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 RP2 in samples. An antibody specific for RP2 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyRP2 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for RP2 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 RP2 bound in the initial step. The color development is stopped and the intensity of the color is measured.

39,641 Da

RP2, ARL3 GTPase activating protein

protein XRP2

Protein XRP2

The RP2 locus has been implicated as one cause of X-linked retinitis pigmentosa. The predicted gene product shows homology with human cofactor C, a protein involved in the ultimate step of beta-tubulin folding. Progressive retinal degeneration may therefore be due to the accumulation of incorrectly-folded photoreceptor or neuron-specific tubulin isoforms followed by progressive cell death [provided by RefSeq, Jul 2008]

RP2: Acts as a GTPase-activating protein (GAP) involved in trafficking between the Golgi and the ciliary membrane. Involved in localization of proteins, such as NPHP3, to the cilium membrane by inducing hydrolysis of GTP ARL3, leading to the release of UNC119 (or UNC119B). Acts as a GTPase-activating protein (GAP) for tubulin in concert with tubulin-specific chaperone C, but does not enhance tubulin heterodimerization. Acts as guanine nucleotide dissociation inhibitor towards ADP-ribosylation factor-like proteins. Defects in RP2 are the cause of retinitis pigmentosa type 2 (RP2); also known as X-linked retinitis pigmentosa 2 (XLRP-2). RP leads to degeneration of retinal photoreceptor cells. 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. Belongs to the TBCC family.

Protein type: Chaperone

Chromosomal Location of Human Ortholog: Xp11.3

Cellular Component: centriole; cilium; cytoplasm; cytoplasmic vesicle; Golgi apparatus; nuclear body; nucleoplasm; plasma membrane

Molecular Function: actin binding; GTP binding; GTPase activator activity; protein binding; unfolded protein binding

Biological Process: cytoskeleton organization; positive regulation of GTPase activity; post-chaperonin tubulin folding pathway; post-Golgi vesicle-mediated transport; protein folding; protein transport; visual perception

Disease: Retinitis Pigmentosa 2

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