KEY RESOURCES TABLE
| REAGENT or RESOURCE | SOURCE | IDENTIFIER |
|---|---|---|
| Antibodies | ||
| Mouse anti-FLAG | Sigma-Aldrich | F1804 |
| Rabbit anti-FLAG | Sigma-Aldrich | F7425 |
| Chicken anti-GFP | Abcam | ab13970 |
| Mouse anti-TOM20 | Santa Cruz Biotech | sc17764 |
| Mouse anti-Opa1 | BD Biosciences | 612806 |
| Mouse anti-Tom40 | Santa Cruz Biotech | sc365467 |
| Mouse anti-Tom70 | Santa Cruz Biotech | sc390545 |
| Mouse anti-VDAC1 | Santa Cruz Biotech | sc390996 |
| Rabbit anti-cytochrome C | Abcam | ab90529 |
| Rabbit anti-C-I30 | Abcam | ab14711 |
| Mouse anti-Core2 | Santa Cruz Biotech | sc390378 |
| Mouse anti-alpha-tubulin | Proteintech | 66031 |
| Rabbit anti-Grp75 | Cell Signaling | 2816 |
| Mouse anti-Hsp60 | Santa Cruz | sc-59567 |
| Mouse anti-C-IV s.1 | Abcam | ab14705 |
| Rat anti-HA | Roche | 3F10 |
| Mouse anti-actin | Sigma-Aldrich | A2228 |
| Rabbit anti-mitofilin | Abcam | ab48139 |
| Rabbit anti-CHCHD3 | Abcam | ab98975 |
| Mouse anti-ApooL | Santa Cruz Biotech | sc-390958 |
| Mouse anti-LETM1 | Abcam | ab55434 |
| Mouse anti-Myc | Santa Cruz Biotech | 9E10 |
| Rabbit anti-Minos1 | Abcam | ab84969 |
| Rabbit anti-fly-Opa1 | Sigma-Aldrich | M6319 |
| Rat anti-poly (GR) | Millipore | MABN778 |
| Rabbit anti-poly (GR) | Millipore | ABN1361 |
| Rabbit anti-poly(GA) | Proteintech | 24492–1-AP |
| Rabbit anti-poly(PR) | Proteintech | 23979–1-AP |
| Rabbit anti-ATP6 | Abcam | AB102573 |
| Goat anti-Mouse IgG-HRP | Santa Cruz | sc-2005 |
| Goat anti-Rabbit IgG HRP | Santa Cruz | sc-2004 |
| Goat anti-Chicken IgY (H+L) Secondary Antibody, Alexa Fluor 488 | Invitrogen | A11039 |
| Goat anti-Rabbit IgG (H+L) Highly Cross-Adsorbed Secondary Antibody, Alexa Fluor 488 | Invitrogen | A11034 |
| Goat anti-Rabbit IgG (H+L) Highly Cross-Adsorbed Secondary Antibody, Alexa Fluor 568 | Invitrogen | A11036 |
| Goat anti-Mouse IgG (H+L) Cross-Adsorbed Secondary Antibody, Alexa Fluor 568 | Invitrogen | A11004 |
| Goat anti-Mouse IgG (H+L) Cross-Adsorbed Secondary Antibody, Alexa Fluor 633 | Invitrogen | A21050 |
| DAPI | Sigma-Aldrich | D9542 |
| Chemicals, Peptides, and Recombinant Proteins | ||
| Nigericin | Cayman Chemical | 11437 |
| Monensin sodium salt | Sigma-Aldrich | M5273 |
| EDC (1-ethyl-3-(3-dimethylaminopropyl) carbodiimide hydrochloride) | Thermo Scientific | 22980 |
| Lipofectamine 3000 | Invitrogen | L3000015 |
| Lipofectamine RNAi-MAX | Invitrogen | 13778150 |
| Tris base | Sigma-Aldrich | 11814273001 |
| Glycine | Sigma-Aldrich | G8898 |
| SDS | Sigma-Aldrich | L3771 |
| Anti-FLAG M2 affinity gel | Sigma-Aldrich | A2220 |
| Normal goat serum | Jackson ImmunoResearch | 005-000-121 |
| Pierce 16% Formaldehyde (w/v), Methanol-free | Thermo Fisher | 28908 |
| Triton™ X-100 | Sigma Aldrich | T9284 |
| DMEM, high glucose, GlutaMAX Supplement | GIBCO | 10566016 |
| Schneider’s Medium | GIBCO | 21720–024 |
| Percoll | GE Healthcare | 17089101 |
| EDTA | Sigma-Aldrich | E9884 |
| EGTA | Sigma-Aldrich | E3889 |
| Dimethyl sulfoxide | Sigma-Aldrich | D8418 |
| 2XLaemmli sample buffer | Biorad | 161–0737 |
| Protease inhibitor cocktail | Bimake | B14012 |
| Critical Commercial Assays | ||
| ATP Bioluminescence Assay Kit HS II | Roche | 11699709001 |
| Complex I Enzyme Activity Microplate Assay Kit | Abcam | ab109721 |
| Protease K digestion kit | Thermo Fisher | AM2548 |
| RNeasy Mini kit | QIAGEN | 74104 |
| iScript cDNA synthesis ki | Biorad | 1708890 |
| PowerUp SYBR Green Master Mix | Applied Biosystems | A25741 |
| Western Lightning Plus-ECL | PerkinElmer Inc. | NEL105001EA |
| HyBlot CL Autoradiography Film | Denville Scientific Inc. | 1159M38 |
| Tetramethylrhodamine (TMRM) | Invitogen | T668 |
| MitoSox | Invitogen | M36008 |
| Rhod-2, AM | Invitogen | R1244 |
| MitoPOP | Dr. Karin Nowikovsky | N/A |
| JC-1 | Invitogen | T3168 |
| Q5 Site-Directed Mutagenesis Kit | New England Biolabs | E0554S |
| NuPAGE® MOPS SDS running buffer | Invitrogen | NP0001 |
| NuPAGE 4–12% Bis-Tris Protein Gels | Invitrogen | NP0321 |
| Oligonucleotides | ||
| qRT-PCR primer: actin42A Forward: TCTTACTGAGCGCGGTTACAG | This paper | N/A |
| qRT-PCR primer: actin42A Reverse: ATGTCGCGCACAATTTCAC | This paper | N/A |
| qRT-PCR primer: mic10 Forward: TCACCCTGCTCTTCTTCCG | This paper | N/A |
| qRT-PCR primer: mic10 Reverse: ACGATATGCCACGCCCAC | This paper | N/A |
| qRT-PCR primer: ApooL Forward: AATCATGGCGGCGGTTGC | This paper | N/A |
| qRT-PCR primer: ApooL Reverse: TGTGCCTTAGCGGTTTCC | This paper | N/A |
| Stealth RNAi siRNA Negative Control Hi GC | Invitrogen | 12935–400 |
| Stealth RNAi™ siRNA of Opa1 | Invitrogen | HSS107432 |
| Stealth RNAi™ siRNA of Letm1 | Invitrogen | HSS106021 |
| Stealth RNAi™ siRNA of Tom40 | Invitrogen | HSS145636 |
| Stealth RNAi™ siRNA of Mitofilin-IMMT | Invitrogen | HSS116992 |
| Stealth RNAi™ siRNA of CHCHD3 | Invitrogen | HSS147816 |
| Stealth RNAi™ siRNA of ApooL | Invitrogen | HSS175195 |
| Stealth RNAi™ siRNA of Minos1 | Invitrogen | AS029RSI |
| Recombinant DNA | ||
| pcDNA3.1-Flag-GR80 | Dr Fen-Biao Gao | N/A |
| pCMV6-C1Orf151 (Minos1)-DDK-Myc | Origene | RC212930 |
| Software and Algorithms | ||
| SPSS | IBM | https://www.ibm.com/analytics/spss-statistics-software |
| Excel | Microsoft | https://www.microsoft.com/en-us/microsoft-365/excel |
| Fiji ImageJ | National Institute of Health | https://imagej.nih.gov/ij/ |
| Deposited Data | ||
| Mendeley Dataset | This Paper | https://data.mendeley.com/datasets/sd3rtdy7sn/1 |
| Experimental Models: Cell Lines | ||
| HEK293 cell line | ATCC | CRC-1573 |
| HeLa cell line | Gift from Dr. Richard Youle | N/A |
| Normal fibroblast cell line #1 | Coriell Institute | ND29510 |
| Normal fibroblast cell line #2 | Coriell Institute | ND29971 |
| C9ALS fibroblast cell line #4 | Gift from Dr. Aaron Gitler | Kramer et al., 2016 |
| C9ALS fibroblast cell line #6 | Gift from Dr. Aaron Gitler | Kramer et al., 2016 |
| C9ALS fibroblast cell line #7 | Gift from Dr. Aaron Gitler | Kramer et al., 2016 |
| Experimental Models: Organisms/Strains | ||
| UAS-Flag-GR80 | Gift from Dr. Fen-Biao Gao | Yang et al., 2015 |
| UAS-Flag-GA80 | Gift from Dr. Fen-Biao Gao | Yang et al., 2015 |
| UAS-Flag-PR80 | Gift from Dr. Fen-Biao Gao | Yang et al., 2015 |
| UAS-mito-GFP | Gift from Dr. William Saxton | N/A |
| UAS-mito-GCaMP | Gift from Dr. Fumiko Kawasaki | N/A |
| UAS-Myc-Mitofilin | Gift from Dr. Xinnan Wang | N/A |
| UAS-dOpa1-Flag, from Dr. Leo Pallanck | Gift from Dr. Leo Pallanck | N/A |
| UAS-dOpa1-RNAi | Gift from Dr. Leo Pallanck | N/A |
| UAS-GR100 | Bloomington Drosophila Stock Center (BDSC) | #58696 |
| UAS-PA100 | BDSC | #58699 |
| UAS-G4C2–36 | BDSC | #58688 |
| UAS-PR100 | BDSC | #58698 |
| UAS-G4C2–36-RNA Only | BDSC | #58689 |
| UAS-PA36 | BDSC | #58695 |
| UAS-PR36 | BDSC | #58694 |
| UAS-GA100 | BDSC | #58697 |
| UAS-GA36 | BDSC | #58693 |
| UAS-GR36 | BDSC | #58692 |
| UAS-Drp1 | BDSC | #51647 |
| UAS-Marf-RNAi | BDSC | #55189 |
| Chchd3 RNAi | BDSC | #51157 |
| Chchd3 RNAi | BDSC | #38984 |
| Chchd3 EP | BDSC | #11599 |
| Mitofilin RNAi | BDSC | #63994 |
| Letm1 RNAi | BDSC | #37502 |
| Tom40 RNAi | BDSC | #26005 |
| Mic10 RNAi | BDSC | #63669 |
| ApooL RNAi | BDSC | #66933 |
| Minos1-HA | FlyORF | F002914 |
| Letm1-HA | FlyORF | F001238 |
| Tim17b RNAi | VDRC | v103529 |