retinitis pigmentosa GTPase regulator, ELISA Kit

Human X-linked retinitis pigmentosa GTPase regulator (RPGR) ELISA kit; COD1; CORDX1; CRD; PCDX; RP15; RP3; XLRP3; orf15; cone dystrophy 1 (X-linked) ; retinitis pigmentosa 15; retinitis pigmentosa 3 GTPase regulator; retinitis pigmentosa GTPase regulator

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

Store all reagents at 2-8 degree C

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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.

MBS9338533 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the 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 MBS9338533 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(%).

113,387 Da

retinitis pigmentosa GTPase regulator

X-linked retinitis pigmentosa GTPase regulator; retinitis pigmentosa 15; retinitis pigmentosa 3 GTPase regulator

X-linked retinitis pigmentosa GTPase regulator

RP3; XLRP3  [Similar Products]

RPGR_HUMAN

This gene encodes a protein with a series of six RCC1-like domains (RLDs), characteristic of the highly conserved guanine nucleotide exchange factors. The encoded protein is found in the Golgi body and interacts with RPGRIP1. This protein localizes to the outer segment of rod photoreceptors and is essential for their viability. Mutations in this gene have been associated with X-linked retinitis pigmentosa (XLRP). Multiple alternatively spliced transcript variants that encode different isoforms of this gene have been reported, but the full-length natures of only some have been determined. [provided by RefSeq, Dec 2008]

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: GEFs; GEFs, Rab

Chromosomal Location of Human Ortholog: Xp21.1

Cellular Component: Golgi apparatus; centrosome; photoreceptor outer segment

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

Biological Process: intracellular protein transport; eye photoreceptor cell development; visual perception; response to stimulus; intraflagellar transport; cilium biogenesis; positive regulation of GTPase activity

Disease: Retinitis Pigmentosa, X-linked, And Sinorespiratory Infections, With Or Without Deafness; Cone-rod Dystrophy, X-linked, 1; Macular Degeneration, X-linked Atrophic; Retinitis Pigmentosa 3

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