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外周蛋白2抗体
  • 产品货号：
    BN40288R
  • 中文名称：
    外周蛋白2抗体
  • 英文名称：
    Rabbit anti-PRPH2 Polyclonal antibody

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  • BN40288R-100ul

    100ul

    ¥2360.00

    交叉反应：Mouse(predicted:Human,Rat,Chicken,Dog,Cow,Horse,Sheep) 推荐应用：WB,IHC-P,IHC-F,ICC,IF,ELISA

  • BN40288R-200ul

    200ul

    ¥3490.00

    交叉反应：Mouse(predicted:Human,Rat,Chicken,Dog,Cow,Horse,Sheep) 推荐应用：WB,IHC-P,IHC-F,ICC,IF,ELISA

英文名称PRPH2
中文名称外周蛋白2抗体
别    名Peripherin-2; PRPH2; PRPH2_HUMAN; Retinal degeneration slow protein; Tetraspanin-22; Tspan-22.  
研究领域神经生物学  信号转导  细胞骨架  细胞膜蛋白  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Mouse,  (predicted: Human, Rat, Chicken, Dog, Cow, Horse, Sheep, )
产品应用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量39kDa
细胞定位细胞膜 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human PRPH2:131-230/346 
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍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.

Function:
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.

Subunit:
Homodimer; disulfide-linked. Probably forms a complex with a ROM1 homodimer. Other proteins could associate with this complex in rods. Interacts with MREG.

Subcellular Location:
Membrane; Multi-pass membrane protein.

Tissue Specificity:
Retina (photoreceptor). In rim region of ROS (rod outer segment) disks.

DISEASE:
Defects in PRPH2 are the cause of retinitis pigmentosa type 7 (RP7). 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.
Defects in PRPH2 are a cause of adult-onset vitelliform macular dystrophy (AVMD). 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). 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). 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.


Similarity:
Belongs to the PRPH2/ROM1 family.

SWISS:
P23942

Gene ID:
5961

Database links:

Entrez Gene: 5961 Human

Entrez Gene: 19133 Mouse

Entrez Gene: 25534 Rat

Omim: 179605 Human

SwissProt: p35906 Cat

SwissProt: P17810 Cow

SwissProt: P52204 Dog

SwissProt: P23942 Human

SwissProt: P15499 Mouse

SwissProt: P17438 Rat

Unigene: 654489 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.





















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