Inter-Species Differences in Regulation of the Progranulin–Sortilin Axis in TDP-43 Cell Models of Neurodegeneration
Abstract
:1. Introduction
2. Results
2.1. Analysis of Intracellular and Secreted Pgrn Protein in Murine TDP-43 LOF and GOF Cell Models
2.2. Analysis of Intracellular and Secreted PGRN Protein in Human TDP-43 LOF and GOF Cell Models
2.3. Analysis of Sortilin Splicing and Protein Levels upon TDP-43 LOF in Murine and Human Neuronal Cell Models
3. Discussion
4. Materials and Methods
4.1. Cell Culture
4.2. Plasmid Constructs and Cell Transfection
4.3. Western Blot Assay
4.4. ELISA Assay
4.5. RNA Isolation, Reverse Transcription (RT) and Real Time PCR
4.6. Immunofluorescence and Image Acquisition
4.7. Nucleotide Sequence Alignment and in silico Analysis
4.8. UV-Cross-Linking and Immunoprecipitation (UV-CLIP)
4.9. Statistical Analyses
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Conflicts of Interest
Abbreviations
ALS | amyotrophic lateral sclerosis |
FTLD | frontotemporal lobar dementia |
RBP | RNA-binding protein |
TDP-43 | TAR DNA binding protein 43 |
PGRN | progranulin |
SORT1 | sortilin 1 |
LOF | loss of function |
GOF | gain of function |
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Gumina, V.; Onesto, E.; Colombrita, C.; Maraschi, A.; Silani, V.; Ratti, A. Inter-Species Differences in Regulation of the Progranulin–Sortilin Axis in TDP-43 Cell Models of Neurodegeneration. Int. J. Mol. Sci. 2019, 20, 5866. https://doi.org/10.3390/ijms20235866
Gumina V, Onesto E, Colombrita C, Maraschi A, Silani V, Ratti A. Inter-Species Differences in Regulation of the Progranulin–Sortilin Axis in TDP-43 Cell Models of Neurodegeneration. International Journal of Molecular Sciences. 2019; 20(23):5866. https://doi.org/10.3390/ijms20235866
Chicago/Turabian StyleGumina, Valentina, Elisa Onesto, Claudia Colombrita, AnnaMaria Maraschi, Vincenzo Silani, and Antonia Ratti. 2019. "Inter-Species Differences in Regulation of the Progranulin–Sortilin Axis in TDP-43 Cell Models of Neurodegeneration" International Journal of Molecular Sciences 20, no. 23: 5866. https://doi.org/10.3390/ijms20235866