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SMN1

SMN1,全稱為運動神經元存活蛋白1,常被簡稱為SMN蛋白,也有SMNt等別名。這種蛋白在人體細胞中扮演著關鍵角色,尤其對運動神經元的存活和正常功能維持至關重要,它參與細胞內多種基礎生命活動,保障神經信號的正常傳遞和肌肉的協調運動。
當SMN1基因出現異常時,SMN蛋白的產量會大幅下降,這是引發脊髓性肌萎縮癥(SMA)的核心原因——SMA是一種罕見的神經肌肉疾病,患者多在嬰幼兒時期發病,表現為進行性肌肉無力、運動發育遲緩,嚴重時會累及呼吸肌導致生命危險。近年來針對SMA的治療取得顯著進展:諾西那生鈉可通過調節RNA剪接提升SMN蛋白水平,利司撲蘭作為口服小分子藥物方便患者長期使用,還有基因治療藥物Zolgensma能直接補充正常的SMN1基因,這些療法極大改善了SMA患者的預后。

熱銷產品

Recombinant Human Survival motor neuron protein (SMN1) (CSB-EP614982HU)

驗證數據

CSB-EP614982HU

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

SMN1 Recombinant Monoclonal Antibody (CSB-RA577775A0HU)

驗證數據

CSB-RA577775A0HU

Flow cytometric analysis of SMN1 expression in HAP-1 cells using SMN1 antibody. Green, isotype control; red, SMN1.

CSB-RA577775A0HU

Immunocytochemical staining of H9c2 cells with SMN1 antibody. Nuclei were stained blue with DAPI; SMN1 was stained magenta with Alexa Fluor? 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: High. Scale bar, 20 μm.

CSB-RA577775A0HU

Western blotting analysis using SMN1 antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with SMN1 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively.

SMN1 Antibody (CSB-PA614982LA01HU)

驗證數據

CSB-PA614982LA01HU

Western Blot
Positive WB detected in: A549 whole cell lysate, Hela whole cell lysate
All lanes: SMN1 antibody at 2µg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 32, 29, 31, 28 kDa
Observed band size: 40, 60 kDa

CSB-PA614982LA01HU

Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA614982LA01HU at dilution of 1:100

CSB-PA614982LA01HU

Immunofluorescent analysis of HepG2 cells using CSB-PA614982LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)

SMN1 Antibodies

SMN1 for Homo sapiens (Human)

SMN1 Proteins

SMN1 Proteins for Homo sapiens (Human)



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