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
(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
Flow cytometric analysis of SMN1 expression in HAP-1 cells using SMN1 antibody. Green, isotype control; red, SMN1.
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.
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
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
Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA614982LA01HU at dilution of 1:100
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)
| 產品貨號 | 產品名稱 | 種屬反應性 | 應用類型 |
|---|---|---|---|
| CSB-PA135133 | SMN1 Antibody | Human | ELISA,WB |
| CSB-PA678622 | SMN1 Antibody | Human | ELISA,WB |
| CSB-PA614982LA01HU | SMN1 Antibody | Human | ELISA, WB, IHC, IF |
| CSB-PA614982LB01HU | SMN1 Antibody, HRP conjugated | Human | ELISA |
| CSB-PA614982LC01HU | SMN1 Antibody, FITC conjugated | Human | |
| CSB-PA614982LD01HU | SMN1 Antibody, Biotin conjugated | Human | ELISA |
| CSB-RA567382A0HU | SMN1 Recombinant Monoclonal Antibody | Human | ELISA, IHC |
| CSB-RA577775A0HU | SMN1 Recombinant Monoclonal Antibody | Human, Mouse, Rat | ELISA, WB, FC, ICC |
SMN1 Proteins
SMN1 Proteins for Homo sapiens (Human)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| CSB-BP614982HU CSB-MP614982HU CSB-EP614982HU-B |
Recombinant Human Survival motor neuron protein (SMN1) | Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
| CSB-EP614982HU | Recombinant Human Survival motor neuron protein (SMN1) | E.coli |
| CSB-YP614982HU | Recombinant Human Survival motor neuron protein (SMN1), partial | Yeast |




