RAN抗体

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     Black line: Control Antigen (100 ng);Purple line: Antigen (10ng); Blue line: Antigen (50 ng); Red line:Antigen (100 ng)    

  
  

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     Western blot analysis using RAN mouse mAb against Hela (1), NIH/3T3 (2), A431 (3), C6 (4), Jurkat (5), Hela (6), COS7 (7), and Jurkat (8) cell lysate.    

  
  

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     Immunofluorescence analysis of GC-7901 cells using RAN mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor- 555 phalloidin. Secondary antibody from Fisher (Cat#: 35503)    

  
  

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     Immunofluorescence analysis of Hela cells using RAN mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor- 555 phalloidin. Secondary antibody from Fisher (Cat#: 35503)    

  
  

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     Immunofluorescence analysis of HepG2 cells using RAN mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor- 555 phalloidin. Secondary antibody from Fisher (Cat#: 35503)    

  
  

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     Flow cytometric analysis of Hela cells using RAN mouse mAb (green) and negative control (red).    

  
  

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     Immunohistochemical analysis of paraffin-embedded human stomach cancer tissues using RAN mouse mAb with DAB staining.    

  
  

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     Immunohistochemical analysis of paraffin-embedded human cervical cancer tissues using RAN mouse mAb with DAB staining.    

  
  

RAN (Repeat Associated Non-AUG) antibodies are a group of autoantibodies identified in neurodegenerative disorders, particularly in conditions linked to nucleotide repeat expansions. They are most notably associated with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) caused by a hexanucleotide repeat expansion (GGGGCC) in the *C9orf72* gene. Unlike traditional antibodies targeting foreign antigens, RAN antibodies recognize aberrant proteins produced through non-canonical translation of expanded RNA repeats, a process termed RAN translation.

In healthy cells, such repetitive sequences are typically not translated into proteins. However, in neurodegenerative diseases, these repeats form secondary RNA structures that recruit ribosomes, initiating translation without an AUG start codon. This generates toxic dipeptide repeat (DPR) proteins (e.g., poly-GA, poly-GR), which aggregate in neurons, disrupting cellular functions and contributing to disease pathology. RAN antibodies specifically detect these misfolded DPR proteins, serving as biomarkers to identify and monitor disease progression.

Their discovery has advanced the understanding of molecular mechanisms in repeat expansion disorders, bridging RNA toxicity and proteinopathy. Clinically, RAN antibodies aid in diagnosing genetic subtypes of ALS/FTD and differentiating them from other neurodegenerative conditions. Research continues to explore their role in therapeutic targeting and prognostic stratification.