KEY RESOURCES TABLE
| REAGENT or RESOURCE Antibodies | SOURCE | IDENTIFIER |
|---|---|---|
| Antibodies | ||
| Mouse monoclonal anti-c-Myc Clone 9E10 | Sigma-Aldrich | Cat#M4439; RRID: AB_439694 |
| Mouse monoclonal anti-FLAG clone M2 | Sigma-Aldrich | Cat#F3165; RRID:AB_259529 |
| Mouse monoclonal anti-GAPDH | Meridian | Cat#H86504M; RRID: AB_151542 |
| Mouse monoclonal anti-HA clone 12CA5 | Roche | Cat# 11583816001; RRID: AB_514505 |
| Mouse monoclonal anti-calbindin-D-28K clone CB-955 | Sigma-Aldrich | Cat#C9848; RRID: AB_476894 |
| Rabbit polyclonal anti-poly(GP) | in-house | Rb5823 |
| Rabbit polyclonal anti-poly(PR) | in-house | Rb8736 |
| Rabbit polyclonal anti-poly(GA) | in-house | Rb9880 |
| Rabbit polyclonal anti-poly(GR) | in-house | Rb7810 |
| Rabbit polyclonal anti-GFAP | Biogenex | Cat#PU020-UP |
| Mouse monoclonal anti-NeuN clone 360 | Millipore | Cat#MAB377; RRID: AB_2298772 |
| Mouse monoclonal Anti-ubiquitin clone Ubi-1 (042691GS) | Chemicon/Millipore | Cat#MAB1510; RRID: AB_2180556 |
| Rabbit polyclonal Anti-SQSTM1/p62 | Cell Signaling | Cat#5114S; RRID:AB_10624872 |
| Anti-pTDP-43 pS409/410 | in-house | Rb3655 |
| Rabbit polyclonal Anti-pTDP-43 pS409/410 | Cosmo Bio | Cat#CAC-TIP-PTD-P02; RRID: AB_1961898 |
| Rabbit polyclonal anti-Ataxin2 | Proteintech | Cat#21776–1-AP; RRID: AB_10858483 |
| Anti-eIF3h clone A5342 | Santa Cruz Biotechnology | Cat#sc-16377; RRID: AB_671941 |
| Bacterial and Virus Strains | ||
| pAAV-CBA-C9orf72-(G4C2)2-3Tag | Chew etal. (2015) | N/A |
| pAAV-CBA-C9orf72-(G4C2)66-3Tag | Chew etal. (2015) | N/A |
| pAAV-CBA-NOP56-(TG3C2)6-3Tag | This study. | N/A |
| pAAV-CBA-NOP56-(TG3C2)62-3Tag | This study. | N/A |
| Biological Samples | ||
| SCA36 patient-derived cortex and cerebellum | Biobank Galicia Sur Health Research Institute | https://www.iisgaliciasur.es/home/biobank-iisgs/?lang=en |
| c9FTD/ALS patient-derived cortex and cerebellum | Emory Neuropathology Core | http://neurology.emory.edu/ENNCF/neuropathology |
| Experimental Models: Cell Lines | ||
| HEK293T | Laboratory of Dr. Petrucelli; ATCC | Cat#CRL-3216; RRID: CVCL_0063 |
| Experimental Models: Organisms/Strains | ||
| Mice: strain C57BL/6J | Laboratory of Dr. Petrucelli; The Jackson Laboratory | Cat# JAX:000664; RRID:IMSR_JAX:000664 |
| Oligonucleotides | ||
| LNA probe: SCA36 sense: 5TYE563- CTGGGCCTGGGCCTG | Exiqon | ID#300500; batch #620467 |
| LNA probe: SCA36 antisense: 5TYE563- AGGCCCAGGCCCAG | Exiqon | ID#300500; batch# 562515 |
| SCA36 qPCR Forward Primer: GTGGTTGCGGGGCGACGC | Integrated DNA Technologies | N/A |
| SCA36 qPCR Reverse Primer: GGCTCTGTCTGCGGCCCG | Integrated DNA Technologies | N/A |
| WPRE qPCR Forward Primer: GGCTGTTGGGCACTGACAAT | Integrated DNA Technologies | N/A |
| WPRE qPCR Reverse Primer: CCGAAGGGACGTAGCAGAAG | Integrated DNA Technologies | N/A |
| Software and Algorithms | ||
| Zeiss AxioVision v4.9.10 | Carl Zeiss Microscopy | https://www.micro-shop.zeiss.com/en/us/system/software-axiovision+software-products/1007/ |
| Aperio eSlide Manager v12.4.2.5010 | Leica Biosystems | https://www.leicabiosystems.com/aperio-eslide-manager/ |
| Aperio ImageScope v12.4.2.7000 | Leica Biosystems | https://www.leicabiosystems.com/digital-pathology/manage/aperio-imagescope/ |
| Graphpad Prism v8.1.1 | GraphPad | https://www.graphpad.com/scientific-software/prism/ |