概述

  • 产品名称Anti-RPGR抗体
    参阅全部 RPGR 一抗
  • 描述
    兔多克隆抗体to RPGR
  • 经测试应用适用于: IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    antigen: corresponding to internal sequence amino acids 379-509 of the Human RPGR protein (Q92834).

  • 阳性对照
    • Human liver tissue.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • 存储溶液pH: 7.20
    Preservative: 0.02% Sodium azide
    Constituents: 59% PBS, 40% Glycerol
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

相关产品

应用

Our Abpromise guarantee covers the use of ab121143 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
IHC-P 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. Use buffer at pH 6 for antigen retrieval.

靶标

  • 功能Could be a guanine-nucleotide releasing factor.
  • 组织特异性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.
  • 疾病相关Defects in RPGR are the cause of retinitis pigmentosa type 3 (RP3) [MIM:300029]; also known as X-linked retinitis pigmentosa 3 (XLRP-3) or retinitis pigmentosa type 15 (RP15). A X-linked retinal dystrophy belonging to the group of pigmentary retinopathies. RP is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. 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. In RP3, affected males have a severe phenotype, and carrier females show a wide spectrum of clinical features ranging from completely asymptomatic to severe retinitis pigmentosa. Heterozygous women can manifest a form of choroidoretinal degeneration which is distinguished from other types by the absence of visual defects in the presence of a brilliant, scintillating, golden-hued, patchy appearance most striking around the macula, called a tapetal-like retinal reflex.
    Defects in RPGR are the cause of retinitis pigmentosa with deafness and sinorespiratory infections (RPDSI) [MIM:300455]. RPDSI is characterized by the association of primary ciliary dyskinesia and Usher syndrome features. The phenotype has similarities with primary ciliary dyskinesia and Usher syndrome.
    Defects in RPGR are the cause of cone-rod dystrophy X-linked type 1 (CORDX1) [MIM:304020]; also known as cone dystrophy 1 (CO1). CORDs are inherited retinal dystrophies belonging to the group of pigmentary retinopathies. CORDs are characterized by retinal pigment deposits visible on fundus examination, predominantly in the macular region, and initial loss of cone photoreceptors followed by rod degeneration. This leads to decreased visual acuity and sensitivity in the central visual field, followed by loss of peripheral vision. Severe loss of vision occurs earlier than in retinitis pigmentosa. In CORDX1 the degree of rod-photoreceptor involvement can be variable, with degeneration increasing as the disease progresses. Affected individuals (essentially all of whom are males) present with decreased visual acuity, myopia, photophobia, abnormal color vision, full peripheral visual fields, decreased photopic electroretinographic responses, and granularity of the macular retinal pigment epithelium. Although penetrance appears to be nearly 100%, there is variable expressivity with respect to age at onset and severity of symptoms.
    Defects in RPGR are a cause of macular degeneration X-linked atrophic (MDXLA) [MIM:300834]. MDXLA is an ocular disorder characterized by macular atrophy causing progressive loss of visual acuity with minimal peripheral visual impairment. Some patients manifest extensive macular degeneration plus peripheral loss of retinal pigment epithelium and choriocapillaries. Full-field electroretinograms (ERGs) show normal cone and rod responses in some affected males despite advanced macular degeneration.
  • 序列相似性Contains 6 RCC1 repeats.
  • 翻译后修饰Prenylated.
  • 细胞定位Golgi apparatus.
  • Information by UniProt
  • 数据库链接
  • 别名
    • COD1 antibody
    • Cone Dystrophy 1 (X-Linked) antibody
    • CORDX1 antibody
    • CRD antibody
    • Orf15 antibody
    • PCDX antibody
    • Retinitis Pigmentosa 15 antibody
    • Retinitis Pigmentosa GTPase Regulator antibody
    • RP15 antibody
    • RP3 antibody
    • RPGR antibody
    • RPGR_HUMAN antibody
    • X-linked retinitis pigmentosa GTPase regulator antibody
    • XLRP3 antibody
    see all

Anti-RPGR antibody 图像

  • Immunohistochemical staining of Human liver with ab121143 at 1/200 dilution.

Anti-RPGR antibody (ab121143)参考文献

ab121143 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab121143.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"