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外周蛋白2抗體
  • 產(chǎn)品貨號(hào):
    BN40288R
  • 中文名稱:
    外周蛋白2抗體
  • 英文名稱:
    Rabbit anti-PRPH2 Polyclonal antibody
  • 品牌:
    Biorigin
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  • BN40288R-100ul

    100ul

    ¥2360.00

    交叉反應(yīng):Mouse(predicted:Human,Rat,Chicken,Dog,Cow,Horse,Sheep) 推薦應(yīng)用:WB,IHC-P,IHC-F,ICC,IF,ELISA

  • BN40288R-200ul

    200ul

    ¥3490.00

    交叉反應(yīng):Mouse(predicted:Human,Rat,Chicken,Dog,Cow,Horse,Sheep) 推薦應(yīng)用:WB,IHC-P,IHC-F,ICC,IF,ELISA

產(chǎn)品描述

英文名稱PRPH2
中文名稱外周蛋白2抗體
別    名Peripherin-2; PRPH2; PRPH2_HUMAN; Retinal degeneration slow protein; Tetraspanin-22; Tspan-22.  
研究領(lǐng)域神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞骨架  細(xì)胞膜蛋白  
抗體來(lái)源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Mouse,  (predicted: Human, Rat, Chicken, Dog, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)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 (石蠟切片需做抗原修復(fù))
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量39kDa
細(xì)胞定位細(xì)胞膜 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human PRPH2:131-230/346 
亞    型IgG
純化方法affinity purified by Protein A
儲(chǔ) 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹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.