Anti-RPGRIP1 (C15-1317-443)

General description

X-linked retinitis pigmentosa GTPase regulator-interacting protein 1 (UniProt: Q9EPQ2; also known as RPGR-interacting protein 1; RPGRIP1) is encoded by the Rpgrip1 gene (Gene ID 77945) in mouse species. RPGRIP is expressed in rod and cone photoreceptors and is situated between the axonemal microtubules and the plasma membrane. It consists of an N-terminal region that forms coiled coil structures linked to a C-terminal tail that binds retinitis pigmentosa GTPase regulator (RPGR). RPGRIP1 associates with ciliary axoneme and may function as a scaffolding protein that anchors regulatory complexes, including RPGR. RPGRIP is considered to be essential for RPGR function and is involved in normal disk morphogenesis. Lack of RPGRIP leads to diffusely mislocalized RPGR in photoreceptors and defects in disk formation. Defects in RPGR and RPGRIP are known to cause retinitis pigmentosa and Leber congenital amaurosis (LCA) that leads to retinal dystrophy and loss of vision. Ref: Hong D-H., et al (2001). J. Biol. Chem. 276(15). 12091 12099. Zhao, Y., et al. (2003). Proc. Natl. Acad. Sci. USA 100 (7), 3965-3970.

Specificity

This polyclonal antibody detects RPGRIP1 protein in mouse retina and U2OS cells.

Immunogen

Epitope: C-terminus

GST-tagged recombinant fragment corresponding to a sequence in the the C-terminal half of mouse RPGRIP.

Application

Anti-RPGRIP1, Cat. No. ABN1700, is a highly specific rabbit polyclonal antibody that targets X-linked retinitis pigmentosa GTPase regulator-interacting protein 1 and has been tested in Immunofluorescence, Immunocytochemistry, and Western Blotting.

Immunofluorescence Analysis: A representative lot detected RPGRIP1 in retinal sections from WT mice (Zhao, Y., et. al. (2003). Proc Natl Acad Sci U S A. 100(7):3965-70).

Western Blotting Analysis: 1:1,000-1:2,000 of this antibody detected RPGRIP1 in RIPA lysates prepared from mouse retina (Courtesy of Dr. Tiansen at NIH).

Immunofluorescence Analysis: 1:500-1:2,000 of this antibody detected RPGRIP1 in frozen mouse retina (Courtesy of Dr. Tiansen at NIH).

Western Blotting Analysis: A representative lot detected RPGRIP1 in WT and mutant (KO) retinal homogenates (Zhao, Y., et. al. (2003). Proc Natl Acad Sci U S A. 100(7):3965-70).

Immunocytochemistry Analysis: A representative lot detected RPGRIP1 in retina sections (Zhao, Y., et. al. (2003). Proc Natl Acad Sci U S A. 100(7):3965-70).

Research Category
Neuroscience

Quality

Evaluated by Western Blotting in U2OS cell lysate.

Western Blotting Analysis: 1 µg/mL of this antibody detected RPGRIP1 in 10 µg of U2OS cell lysate.

Target description

~ 147 kDa observed; 152 kDa calculated. Uncharacterized bands may be observed in some lysate(s).

Physical form

Affinity Purified

Format: Purified

Purified rabbit polyclonal antibody in PBS without preservatives with 50% glycerol.

Storage and Stability

Stable for 1 year at -20°C from date of receipt.

Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Other Notes

Concentration: Please refer to lot specific datasheet.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

biological source: rabbit. Quality Level: 100. antibody form: affinity isolated antibody. antibody product type: primary antibodies. clone: polyclonal. species reactivity: mouse, human. technique(s): immunocytochemistry: suitable, immunofluorescence: suitable, western blot: suitable. NCBI accession no.: NP_076368. UniProt accession no.: Q9EPQ2. shipped in: ambient. target post-translational modification: unmodified. Gene Information: mouse ... Rpgrip1(77945). Storage Class Code: 10 - Combustible liquids. WGK: WGK 2.