RP2, Polyclonal Antibody

Protein XRP2; RP2

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

Polyclonal

IgG

Rabbit

Antigen Affinity Purified

Liquid

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-RP2 antibody 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.

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.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunoprecipitation (IP)

Western blot
All lanes:  RP2antibody at 3.81ug/ml
Lane 1 : Jurkat whole cell lysate
Lane 2 : HepG-2 whole cell lysate
Lane 3 : Hela whole cell lysate
Secondary
Goat polyclonal to Rabbit IgG at 1/10000 dilution
Predicted band size: 40 kDa
Observed band size: 40 kDa

Immunohistochemistry of paraffin-embedded human kidney tissue using MBS7044960 at dilution 1:100

Immunoprecipitating RP2 in Hela whole cell lysate
Lane 1: Rabbit monoclonal IgG(1ug) instead of MBS7044960 in Hela whole cell lysate. For western blotting, a HRP-conjugated light chain specific antibody was used as the Secondary antibody (1/50000)
Lane 2: MBS7044960(4ug)+ Hela whole cell lysate(500ug)
Lane 3: Hela whole cell lysate (20ug)

39,641 Da

retinitis pigmentosa 2 (X-linked recessive)

protein XRP2

Protein XRP2

XRP2_HUMAN

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: cytoplasm; cytoplasmic vesicle; plasma membrane

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

Biological Process: post-chaperonin tubulin folding pathway; post-Golgi vesicle-mediated transport; protein folding; visual perception

Disease: Retinitis Pigmentosa 2

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