Mouse RP2 Monoclonal Antibody | anti-RP2 antibody

RP2 (Retinitis Pigmentosa 2 (X-Linked Recessive), DELXp11.3, KIAA0215, TBCCD2) (APC)

Cat. Num. AAA6168210

• Gene Names: RP2; XRP2; NME10; TBCCD2; NM23-H10; DELXp11.3
• Applications: Immunofluorescence, Western Blot
• Purity: Purified
• Synonyms: RP2; Monoclonal Antibody; RP2 (Retinitis Pigmentosa 2 (X-Linked Recessive); DELXp11.3; KIAA0215; TBCCD2) (APC); Retinitis Pigmentosa 2 (X-Linked Recessive); TBCCD2; anti-RP2 antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 1B4
• Specificity: Recognizes RP2.
• Purity/Purification: Purified
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

• Applicable Applications for anti-RP2 antibody: Immunofluorescence (IF), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: RP2 (NP_008846, 251aa-350aa) partial recombinant protein with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: RKLIDEMVGKGFFLVQTKEVSMKAEDAQRVFREKAPDFLPLLNKGPVIALEFNGDGAVEVCQLIVNEIFNGTKMFVSESKETASGDVDSFYNFADIQMGI
• Conjugate: APC
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(RP2 monoclonal antibody (M01), clone 1B4. Western Blot analysis of RP2 expression in Hela S3 NE.)

Testing Data

(Detection limit for recombinant GST tagged RP2 is approximately 0.1ng/ml as a capture antibody.)

Immunofluorescence (IF)

(Immunofluorescence of monoclonal antibody to RP2 on HeLa cell. [antibody concentration 10 ug/ml])

Immunofluorescence (IF)

(Immunofluorescence of monoclonal antibody to RP2 on HeLa cell. [antibody concentration 10 ug/ml])

Related Product Information for anti-RP2 antibody

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]

Product Categories/Family for anti-RP2 antibody

Antibodies; Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 170016081
• NCBI GeneID: 6102
• NCBI Accession #: NP_008846
• NCBI GenBank Nucleotide #: NM_006003
• UniProt Accession #: O75695
• Molecular Weight: 40kDa
• NCBI Official Full Name: protein XRP2
• NCBI Official Synonym Full Names: RP2 activator of ARL3 GTPase
• NCBI Official Symbol: RP2
• NCBI Official Synonym Symbols: XRP2; NME10; TBCCD2; NM23-H10; DELXp11.3
• NCBI Protein Information: protein XRP2
• UniProt Protein Name: Protein XRP2
• Protein Family: Protein
• UniProt Gene Name: RP2
• UniProt Entry Name: XRP2_HUMAN

NCBI Description

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]

Uniprot Description

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: centriole; Golgi apparatus; cytoplasm; plasma membrane; cytoplasmic vesicle

Molecular Function: protein binding; GTP binding; unfolded protein binding; nucleoside diphosphate kinase activity; actin binding; GTPase activator activity; ATP binding

Biological Process: GTP biosynthetic process; CTP biosynthetic process; protein transport; protein folding; visual perception; organelle organization and biogenesis; UTP biosynthetic process; cell morphogenesis; nucleoside diphosphate phosphorylation; cytoskeleton organization and biogenesis; post-Golgi vesicle-mediated transport; post-chaperonin tubulin folding pathway; positive regulation of GTPase activity

Disease: Retinitis Pigmentosa 2