产品名称
RPGR Rabbit Polyclonal Antibody (C-term)
别名
X-linked retinitis pigmentosa GTPase regulator, RPGR, RP3, XLRP3
Human Gene ID
NP_000319.1;NP_001030025.1
Human Swissprot No.
Q92834
特异性
This RPGR antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 744-772 amino acids from the C-terminal region of human RPGR.
背景介绍
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.
组织表达
Heart, brain, placenta, lung, liver, muscle, kidney, retina, pancreas and fetal retinal pigment epithelium. Isoform 3 is found only in the retina. Colocalizes with RPGRIP1 in the outer segment of rod photoreceptors and cone outer segments
细胞定位
Cytoplasm, cytoskeleton, flagellum axoneme {ECO:0000250|UniProtKB:Q9R0X5}. Golgi apparatus. Cell projection, cilium {ECO:0000250|UniProtKB:Q9R0X5}. Note=In the retinal photoreceptor cell layer, localizes at the connecting cilium (By similarity). Colocalizes with WHRN in the photoreceptor connecting cilium (By similarity) Colocalizes with CEP290 in the photoreceptor connecting cilium (By similarity). Colocalizes with RPGRIP1 in the photoreceptor connecting cilium (By similarity). {ECO:0000250|UniProtKB:Q9R0X5}
纯化
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
