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PLP1

PLP1,全稱為髓鞘蛋白脂蛋白1,常見別名包括PLP和DM20(后者是其剪接異構(gòu)體)。這種蛋白是中樞神經(jīng)系統(tǒng)內(nèi)少突膠質(zhì)細(xì)胞合成的髓鞘核心成分,對(duì)維持髓鞘的結(jié)構(gòu)完整與功能穩(wěn)定至關(guān)重要,能幫助神經(jīng)信號(hào)在神經(jīng)元間快速傳遞。當(dāng)PLP1出現(xiàn)異常時(shí),易引發(fā)Pelizaeus-Merzbacher病(PMD)——一種X連鎖遺傳的白質(zhì)腦病,患者常表現(xiàn)出運(yùn)動(dòng)發(fā)育遲緩、肢體僵硬、眼球震顫等癥狀,嚴(yán)重影響生活質(zhì)量。目前針對(duì)PMD尚無根治手段,臨床多采用康復(fù)訓(xùn)練等支持療法;不過科研領(lǐng)域已有進(jìn)展,例如反義寡核苷酸藥物正嘗試糾正PLP1的異常剪接,基因治療也在探索通過載體遞送正?;蚋纳瓢Y狀,這些療法仍處于臨床試驗(yàn)階段,為患者帶來了潛在希望。

熱銷產(chǎn)品

Recombinant Human Myelin proteolipid protein (PLP1) (CSB-CF018202HU)

驗(yàn)證數(shù)據(jù)

CSB-CF018202HU

(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

PLP1 Antibody (CSB-PA018202LA01HU)

驗(yàn)證數(shù)據(jù)

CSB-PA018202LA01HU

Western blot
All lanes: PLP1 antibody at 4µg/ml
Lane 1: Hela whole cell lysate
Lane 2: HL60 whole cell lysate
Lane 3: K562 whole cell lysate
Lane 4: THP-1 whole cell lysate
Lane 5: U937 whole cell lysate
Lane 6: A549 whole cell lysate
Lane 7: Caco-2 whole cell lysate
Lane 8: HepG2 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 31, 27 kDa
Observed band size: 31 kDa

CSB-PA018202LA01HU

Immunohistochemistry of paraffin-embedded human breast cancer using CSB-PA018202LA01HU at dilution of 1:100

CSB-PA018202LA01HU

Immunofluorescent analysis of Hela cells using CSB-PA018202LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)

PLP1 Antibodies

PLP1 for Homo sapiens (Human)

PLP1 Proteins

PLP1 Proteins for Homo sapiens (Human)



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