General Notes
This product (Catalog No. DPABH-09720) is a rabbit-derived polyclonal antibody raised against human RP2, targeting the amino acid region 36–350 of the protein. It has been affinity purified by Protein A. The RP2 gene, located on the short arm of the X chromosome at cytogenetic band Xp11.3, encodes a protein known as RP2 (also called XRP2), which acts as a GTPase-activating protein (GAP) for the small GTPase ARL3 and is essential for the intracellular transport of lipid-modified proteins, particularly in photoreceptor cells of the retina. The RP2 protein is expressed ubiquitously across human tissues, with highest levels in bone marrow and placenta, and it localizes to cellular structures including the Golgi apparatus, centrioles, ciliary basal bodies, and plasma membrane, where it may also contribute to tubulin folding and cell motility regulation. Mutations in RP2 are the primary cause of X-linked retinitis pigmentosa type 2 (RP2; OMIM #312600), an early-onset form of retinal dystrophy that accounts for 8–20% of X-linked retinitis pigmentosa cases and leads to progressive photoreceptor degeneration, night blindness starting in childhood, visual field constriction, and eventual severe vision loss or blindness, predominantly in males due to its X-linked recessive inheritance. Over 70 mutations have been identified, including nonsense, frameshift, missense, and splice-site variants that typically result in truncated, unstable, or mislocalized RP2 protein, disrupting photoreceptor maintenance and ciliary function. Female carriers often show mild or no symptoms due to X-chromosome inactivation, though some exhibit fundus abnormalities, reduced electroretinogram amplitudes, or even progressive retinal changes in cases of skewed inactivation.
DPABH-09720 is suitable for use in ELISA (1:4,000–1:8,000), IHC (1:50–1:100), Western blot (WB) (1:1,000–1:4,000) applications.
DPABH-09720 is supplied as a liquid formulation in 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. For long-term storage, keep at −20 °C. For short-term use, store at 2–8 °C. Avoid repeated freeze–thaw cycles to maintain antibody integrity and performance.
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