RP2 recombinant protein

RP2 Protein, Human, Recombinant (GST Tag)

Cat. Num. AAA8120680

• Gene Names: RP2; XRP2; NME10; TBCCD2; NM23-H10; DELXp11.3
• Purity: >95% as determined by SDS-PAGE
• Synonyms: RP2; RP2 Protein; Human; Recombinant (GST Tag); Human XRP2/RP2 Protein (GST Tag); DELXp11.3 Protein; KIAA0215 Protein; NM23-H10 Protein; NME10 Protein; TBCCD2 Protein; XRP2 Protein; retinitis pigmentosa 2 (X-linked recessive); RP2 recombinant protein

• Host: Baculovirus-Insect Cells
• Purity/Purification: >95% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile 50mM Tris, 100mM NaCl, 1mM GSH 0.5mM EDTA, 0.5mM PMSF pH8.0. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of
• Sequence: Met1-Thr350

• Species: Human
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Met
• Tag: N-GST
• Protein Construction: A DNA sequence encoding the human XRP2 (NP_008846.2)(Met 1-Thr 350) was fused with the GST tag at the N-terminus.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Related Product Information for RP2 recombinant protein

Background: XRP2, also known as Protein XRP2 and RP2, is a member of the TBCC (tubulin cofactor C) family and contains one C-CAP/cofactor C-like domain. This protein is encoded by the RP2 gene in humans. XRP2 stimulates the GTPase activity of tubulin, but does not enhance tubulin heterodimerization. XRP2 acts as guanine nucleotide dissociation inhibitor for ARL3. Defects in RP2 gene are the cause of retinitis pigmentosa type 2 (RP2), also known as X-linked retinitis pigmentosa 2 (XLRP-2). It 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.

References

Schwahn U., et al.,(1998), Positional cloning of the gene for X-linked retinitis pigmentosa 2. Nat. Genet. 19:327-332.Ross M.T., et al., (2005), The DNA sequence of the human X chromosome.Nature 434:325-337.Vaca Jacome A.S., et al.,(2015), N-terminome analysis of the human mitochondrial proteome. Proteomics 15:2519-2524.

NCBI and Uniprot Product Information

• NCBI GI #: 170016081
• NCBI GeneID: 6102
• NCBI Accession #: NP_008846.2
• NCBI GenBank Nucleotide #: NM_006915.2
• UniProt Accession #: O75695; Q86XJ7; Q9NU67
• Molecular Weight: 39,641 Da
• NCBI Official Full Name: protein XRP2
• NCBI Official Synonym Full Names: RP2, ARL3 GTPase activating protein
• 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