XRP2 / RP2 recombinant protein

Recombinant Human XRP2 / RP2 Protein (GST tag)

Cat. Num. AAA2545919

• Gene Names: RP2; XRP2; NME10; TBCCD2; NM23-H10; DELXp11.3
• Purity: > 95 % as determined by SDS-PAGE
• Synonyms: XRP2 / RP2; Recombinant Human XRP2 / RP2 Protein (GST tag); RP2; DELXp11.3; KIAA0215; NME10; TBCCD2; XRP2; XRP2 / 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 pH 8.0
• Sequence Length: 350

• Application Notes: The recombinant human XRP2/GST chimera consists of 575 amino acids and predicts a molecular mass of 66 kDa as estimated in SDS-PAGE under reducing conditions.
• Predicted N Terminal: Met
• Endotoxin: < 1.0 EU per mug of the protein as determined by the LAL method
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -70 degree C

SDS-PAGE

Related Product Information for XRP2 / 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. 

Description: A DNA sequence encoding the human XRP2 (NP_008846.2) (Met 1-Thr 350) was fused with the GST tag at the N-terminus.

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: retinitis pigmentosa 2 (X-linked recessive)
• 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
• UniProt Gene Name: RP2

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: 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