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Rabbit Monoclonal PRPF31 Antibody

  • 中文名: PRPF31抗体
  • 别    名: hPrp31; Protein 61K; PRP31; prpf31; RP11; SNRNP61;;PRPF31
货号: IPDX17888
Price: ¥1280
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 1/50-1/200 Human,Mouse,Rat
FCM 1/20-1/100 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliaseshPrp31; Protein 61K; PRP31; prpf31; RP11; SNRNP61;;PRPF31
WB Predicted band sizeCalculated MW: 55 kDa ; Observed MW: 60 kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman
ImmunogenA synthesized peptide derived from human PRPF31
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

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参考文献

以下是关于PRPF31抗体的3篇参考文献(简要概括内容):

1. **"PRPF31 mutations cause autosomal dominant retinitis pigmentosa via haploinsufficiency"**

*作者:Vithana et al. (2001)*

**摘要**:研究通过免疫荧光和Western blot分析,利用PRPF31抗体证实了其在视网膜中的表达,发现PRPF31基因突变导致蛋白功能不足,引发视网膜色素变性。

2. **"Interactions of the human SF3a splicing complex with PRPF31 and its role in pre-mRNA splicing"**

*作者:Liu et al. (2017)*

**摘要**:通过免疫共沉淀(使用PRPF31抗体)和质谱分析,揭示了PRPF31在剪接体复合物中的关键作用,并证明其突变可能破坏mRNA剪接过程。

3. **"Therapeutic rescue of PRPF31-associated retinal degeneration by AAV-mediated gene augmentation"**

*作者:Tanner et al. (2020)*

**摘要**:研究使用PRPF31抗体检测腺相关病毒(AAV)递送的PRPF31基因在视网膜细胞中的表达,发现基因治疗可部分恢复突变导致的蛋白缺陷和视网膜功能。

4. **"PRPF31 deficiency leads to impaired ribosome biogenesis and retinal cell death"**

*作者:Rose et al. (2021)*

**摘要**:通过Western blot和免疫组化(基于PRPF31抗体),发现PRPF31缺失影响核糖体生物合成,导致光感受器细胞凋亡,解释了其在视网膜疾病中的病理机制。

(注:以上文献信息为示例,实际引用需核对真实文献。)

背景信息

**Background of PRPF31 Antibody**

PRPF31 (pre-mRNA processing factor 31) is a core component of the spliceosome, a large ribonucleoprotein complex essential for pre-mRNA splicing. It plays a critical role in recognizing the 3' splice site during the assembly of the U4/U6.U5 tri-snRNP complex, ensuring accurate removal of introns and proper mRNA maturation. Mutations in the *PRPF31* gene are linked to autosomal dominant retinitis pigmentosa (adRP), a hereditary retinal degenerative disease causing progressive vision loss. The pathogenicity is attributed to haploinsufficiency, where reduced protein levels impair spliceosomal function, particularly in stress-prone retinal cells.

PRPF31 antibodies are essential tools for studying the protein's expression, localization, and interaction networks. They are widely used in techniques like Western blotting, immunofluorescence, and immunoprecipitation to investigate PRPF31's role in splicing mechanisms and disease pathology. These antibodies help identify tissue-specific expression patterns, quantify protein levels in disease models, and validate CRISPR-edited or siRNA-treated cells. Commercially available PRPF31 antibodies are typically raised against epitopes within conserved regions, with validation across species (e.g., human, mouse). Their application extends to clinical research, aiding in understanding retinal degeneration mechanisms and exploring therapeutic strategies for *PRPF31*-linked adRP.

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