HPA001593
抗-RPGR 兔抗
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
别名:
抗-X连锁视网膜色素变性 GTP 酶调节因子 兔抗
产品名称
抗-RPGR 兔抗, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
technique(s)
immunohistochemistry: 1:50-1:200
immunogen sequence
EINDTCLSVATFLPYSSLTSGNVLQRTLSARMRRRERERSPDSFSMRRTLPPIEGTLGLSACFLPNSVFPRCSERNLQESVLSEQDLMQPEEPDYLLDEMTKEAEIDNSSTVESLGETTDILNMTHIMSLN
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... RPGR(6103)
Features and Benefits
Prestige Antibodies®是经过高度表征和广泛验证的抗体，同时还有一个优点是其每个靶标的所有可用表征数据都可以通过位于此页面顶部产品名称下方的人类蛋白质图谱门户进行访问。Prestige Antibodies®对其他蛋白质的独特性和低交叉反应性是通过严密的抗原区域选择、亲和纯化和严格的选择来实现的。每种Prestige 抗体都有相应的Prestige 抗原对照品，可在链接部分找到。
每种Prestige 抗体的检测方法如下：
每种Prestige 抗体的检测方法如下：
- 44种正常人体组织和20种最常见癌症组织的IHC组织阵列。
- 364个人重组蛋白片段的蛋白阵列。
Immunogen
X 连锁色素性视网膜炎 GTP 酶调节因子重组蛋白表位特征标签（PrEST）
Other Notes
相应抗原APREST74315
Physical form
磷酸盐缓冲盐水溶液，pH 7.2，含有40%甘油和0.02%叠氮化钠
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Application
兔抗 RPGR 抗体是一种 Prestige 抗体，经人类蛋白质图谱（HPA）计划开发和验证。每种抗体都通过针对数百种正常和疾病组织的免疫组织化学进行测试。通过单击图像库链接，可以在人类蛋白质图谱（HPA）站点上查看这些图像。还采用免疫荧光和蛋白质印迹法对抗体进行检测。想要查看有关Prestige 抗体和HPA的方案和其他实用信息，请访问 sigma.com/prestige。
Biochem/physiol Actions
RPGR（色素性视网膜炎 GTP 酶调节因子）基因编码的蛋白与 Ran GTP 酶的鸟嘌呤核苷酸交换因子具有序列同源性。它在 N 端包含 6 个类似 RCC1 的结构域。它在感光细胞活力的长期维持中起作用。它存在于高尔基体中，并参与与 RPGRIP1（RPGR 相互作用蛋白 1）的相互作用，以介导与囊泡运输相关的过程。这两种蛋白质共定位于视杆细胞的外段。该基因的缺陷与连锁性视网膜色素变性（XLRP）有关。
Disclaimer
除非我们的产品目录或产品附带的其他公司文档另有说明，否则我们的产品仅供研究使用，不得用于任何其他目的，包括但不限于未经授权的商业用途、体外诊断用途、离体或体内治疗用途或任何类型的消费或应用于人类或动物。
未找到合适的产品？
试试我们的产品选型工具.
存储类别
10 - Combustible liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
常规特殊物品
此项目有
M Dominik Fischer et al.
Molecular therapy : the journal of the American Society of Gene Therapy, 25(8), 1854-1865 (2017-05-28)
X-linked retinitis pigmentosa (XLRP) is generally a severe form of retinitis pigmentosa, a neurodegenerative, blinding disorder of the retina. 70% of XLRP cases are due to mutations in the retina-specific isoform of the gene encoding retinitis pigmentosa GTPase regulator (RPGRORF15). Despite
Christine Vössing et al.
International journal of molecular sciences, 22(7) (2021-04-04)
X-linked retinitis pigmentosa (XLRP) is frequently caused by mutations in the retinitis pigmentosa GTPase regulator (RPGR) gene. A complex splicing process acts on the RPGR gene resulting in three major isoforms: RPGRex1-19, RPGRORF15 and RPGRskip14/15. We characterized the widely expressed
Rakesh K Raghupathy et al.
Scientific reports, 7(1), 16881-16881 (2017-12-06)
Mutations in the RPGR-interacting protein 1 (RPGRIP1) gene cause recessive Leber congenital amaurosis (LCA), juvenile retinitis pigmentosa (RP) and cone-rod dystrophy. RPGRIP1 interacts with other retinal disease-causing proteins and has been proposed to have a role in ciliary protein transport;
Milica Gakovic et al.
Human molecular genetics, 20(24), 4840-4850 (2011-09-22)
Mutations in the retinitis pigmentosa GTPase regulator (RPGR) protein cause one of the most common and severe forms of inherited retinal dystrophy. In spite of numerous studies, the precise function of RPGR remains unclear, as is the mechanism by which
Christine Vössing et al.
International journal of molecular sciences, 21(22) (2020-11-14)
X-chromosomal retinitis pigmentosa (RP) frequently is caused by mutations in the retinitis pigmentosa GTPase regulator (RPGR) gene. We evaluated the potential of PTC124 (Ataluren, TranslamaTM) treatment to promote ribosomal read-through of premature termination codons (PTC) in RPGR. Expression constructs in
我们的科学家团队拥有各种研究领域经验，包括生命科学、材料科学、化学合成、色谱、分析及许多其他领域.
联系客户支持