| Products/Services Used | Details | Operation |
|---|---|---|
| Peptide Synthesis | The following primary antibodies were used: α-p62 (Abcam; 1:50), α-ubiquitin (Abcam; 1:400), α- ubiquilin-2 (Abnova; 1:50), α-nucleolin (ProteinTech; 1:200), α-fibrillarin (Abcam; 1:1000), α-poly-GA (ProteinTech; 1:100), α-poly-GR (ProteinTech; 1:100), α-poly-AP (ProteinTech; 1:100), α-poly-GP (ProteinTech; 1:100), α-poly-PR (Genscript; 1:100).... Supplemental Figure 2: Distribution of untagged poly-GA (A), poly-AP (B), poly-GR (C) and poly-PR (D) using specific antibodies against each peptide (ProteinTech or Genscript; green) in HeLa cells, 48h post-transfection. | Get A Quote |
C9orf72 expansions are the most common genetic cause of FTLD and MND identified to date. Although being intronic, the expansion is translated into five different dipeptide repeat proteins (DPRs) that accumulate within patients' neurons. Attempts have been made to model DPRs in cell and animals. However, the majority of these use DPRs repeat numbers much shorter than those observed in patients. To address this we have generated a selection of DPR expression constructs with repeat numbers in excess of 1000 repeats, matching what is seen in patients. Small and larger DPRs produce inclusions with similar morphology but different cellular effects. We demonstrate a length dependent effect using electrophysiology with... More
