X-linked retinitis pigmentosa GTPase regulator is a GTPase-binding protein that in humans is encoded by the RPGR gene. This gene encodes a protein with a series of six RCC1-like domains (RLDs), characteristic of the highly conserved guanine nucleotide exchange factors. The encoded protein is found in the Golgi body and interacts with RPGRIP1. This protein localizes to the outer segment of rod photoreceptors and is essential for their viability. Mutations in this gene have been associated with X-linked retinitis pigmentosa (XLRP). Multiple alternatively spliced transcript variants that encode different isoforms of this gene have been reported, but the full-length natures of only some have been determined.
Formulation
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host
Rabbit
Immunogen Region
A recombinant human protein corresponding to amino acids A13-F338 was used as the immunogen for the RPGR antibody.
Isotype
IgG
Predicted Reactivity
Rat
Reactivity
Rat
Recombinant
No
Subcellular Location
Cytoplasm, cytoskeleton
Antigen
RPGR
Uniprot
Q92834
Buffer
Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Format
Antigen affinity purified
Purification
Antigen affinity purified
Storage
After reconstitution, the RPGR antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
Applications
WB, ELISA
Dilution
Western blot: 0.5-1ug/ml,Direct ELISA: 0.1-0.5ug/ml