概述

  • 产品名称Anti-PRPH2抗体
    参阅全部 PRPH2 一抗
  • 描述
    兔多克隆抗体to PRPH2
  • 经测试应用适用于: WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    antigen, corresponding to amino acids 124-248 of Human PRPH2 (UniProt: P23942).

  • 阳性对照
    • WB: Over-expression Lysate (co-expressed with a C terminal myc-DDK tag (~3.1 kDa) in mammalian HEK293T cells) IHC-P: Human urinary bladder tissue

性能

相关产品

应用

Our Abpromise guarantee covers the use of ab122057 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
WB 1/250 - 1/500.
IHC-P 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

靶标

  • 功能May function as an adhesion molecule involved in stabilization and compaction of outer segment disks or in the maintenance of the curvature of the rim. It is essential for disk morphogenesis.
  • 组织特异性Retina (photoreceptor). In rim region of ROS (rod outer segment) disks.
  • 疾病相关Defects in PRPH2 are the cause of retinitis pigmentosa type 7 (RP7) [MIM:608133]. 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.
    Defects in PRPH2 are a cause of retinitis punctata albescens [MIM:136880].
    Defects in PRPH2 are a cause of adult-onset vitelliform macular dystrophy (AVMD) [MIM:608161]. AVMD is a rare autosomal dominant disorder with incomplete penetrance and highly variable expression. Patients usually become symptomatic in the fourth or fifth decade of life with a protracted disease of decreased visual acuity.
    Defects in PRPH2 are a cause of patterned dystrophy of retinal pigment epithelium (PDREP) [MIM:169150]. Patterned dystrophies of the retinal pigment epithelium (RPE) refer to a heterogeneous group of macular disorders. Three main types of PDREP have been described: reticular (fishnet-like) dystrophy, macroreticular (spider-shaped) dystrophy and butterfly-shaped pigment dystrophy.
    Defects in PRPH2 are a cause of choroidal dystrophy central areolar type 2 (CACD2) [MIM:613105]. It is a disorder which affects the posterior pole of the eye, and early lesions consist of a non-specific area of granular hyperpigmentation at the fovea. The characteristic sign of the disorder, a zone of atrophy that develops in the macula of the eye and involves the retinal pigment epithelium and the choriocapillaris, occurs several decades after onset.
    Note=Defects in PRPH2 are found in different retinal diseases including cone-rod dystrophy, retinitis pigmentosa, macular degeneration. The mutations underlying autosomal dominant retinitis pigmentosa and severe macular degeneration are largely missense or small in-frame deletions in a large intradiscal loop between the third and fourth transmembrane domains. In contrast, those associated with the milder pattern phenotypes or with digenic RP are scattered more evenly through the gene and are often nonsense mutations. This observation correlates with the hypothesis that the large loop is an important site of interaction between PRPH2 molecules and other protein components in the disk.
  • 序列相似性Belongs to the PRPH2/ROM1 family.
  • 细胞定位Membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AOFMD antibody
    • AVMD antibody
    • CACD2 antibody
    • DS antibody
    • Peripherin 2 (retinal degeneration, slow) antibody
    • Peripherin 2, homolog of mouse antibody
    • Peripherin, photoreceptor type antibody
    • Peripherin-2 antibody
    • PRPH antibody
    • PRPH2 antibody
    • PRPH2_HUMAN antibody
    • Rd2 antibody
    • RDS antibody
    • Retinal degeneration slow protein antibody
    • Retinal peripherin antibody
    • RP7 antibody
    • Tetraspanin-22 antibody
    • Tspan-22 antibody
    • TSPAN22 antibody
    see all

Anti-PRPH2 antibody 图像

  • All lanes : Anti-PRPH2 antibody (ab122057) at 1/250 dilution

    Lane 1 : Negative control (vector only transfected HEK293T lysate)
    Lane 2 : Over expression Lysate (co-expressed with a C terminal myc-DDK tag (~3.1 kDa) in mammalian HEK293T cells)

    Developed using the ECL technique
  • ab122057, at 1/250, staining PRPH2 in paraffin embedded Human urinary bladder tissue by Immunohistochemistry.

Anti-PRPH2 antibody (ab122057)参考文献

ab122057 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab122057.
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"