Rabbit anti-Human RPGR Polyclonal Antibody | anti-RPGR antibody

RPGR, CT (RPGR, RP3, XLRP3, X-linked retinitis pigmentosa GTPase regulator)

Cat. Num. AAA642643

• Reactivity: Human
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: RPGR; Polyclonal Antibody; CT (RPGR; RP3; XLRP3; X-linked retinitis pigmentosa GTPase regulator); Anti -RPGR; anti-RPGR antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-RPGR antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Suitable for use in Western Blot, Immunohistochemistry, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500; Immunohistochemistry: 1:10-50
• Immunogen: RPGR antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 743-772 amino acids from the C-terminal region of human RPGR.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in A549 cell line lysates (35ug/lane), using MBS642643. This demonstrates that MBS642643 detected the RPGR protein (arrow).)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis in formalin fixed and paraffin embedded human liver tissue, using MBS642643 followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of MBS642643 for immunohistochemistry.)

Related Product Information for anti-RPGR antibody

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.

Product Categories/Family for anti-RPGR antibody

Antibodies; Abs to Disease Markers

NCBI and Uniprot Product Information

• NCBI GI #: 5823976
• UniProt Accession #: Q92834; O00702; O00737; Q3KN84; Q8N5T6; Q93039; Q9HD29; Q9UMR1; B1ARN3; E9PE28
• Molecular Weight: 113,387 Da
• NCBI Official Full Name: RPGR protein
• UniProt Protein Name: X-linked retinitis pigmentosa GTPase regulator
• Protein Family: X-linked retinitis pigmentosa GTPase regulator
• UniProt Gene Name: RPGR
• UniProt Synonym Gene Names: RP3; XLRP3
• UniProt Entry Name: RPGR_HUMAN

Uniprot Description

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, Rab; GEFs

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