MTT Assay:Article Title: Targeting PGAM5-driven mitochondrial integrated stress response slows ALS progression across subtypes.
Article Snippet: REAGENT or RESOURCE SOURCE IDENTIFIER Mouse monoclonal anti-MAP2 Sigma Cat# M1406; RRID:AB_477171 Mouse monoclonal anti-HB9 DSHB Cat# 81.5C10; RRID:AB_2145209 Mouse monoclonal anti-ISL1 DSHB Cat# 40.2D6; RRID:AB_528315 Rabbit polyclonal anti-BIP HUABIO Cat# ER1706-50; RRID:AB_3069065 Rabbit polyclonal anti-ATP5B Proteintech Cat# 17247-1-AP; RRID:AB_2061878 Rabbit polyclonal anti-MFN1 Proteintech Cat# 13798-1-AP; RRID:AB_2266318 Rabbit Monoclonal anti-ATF4 CST Cat# 11815; RRID:AB_2616025 Goat Anti-Rabbit IgG H&L Novoprotein Cat# N269; RRID:AB_3697628 Goat polyclonal anti-OLIG2 Santa Cruz Cat# sc-19969; RRID:AB_2236477 Bacterial and virus strains E.coli DH5α TIANGEN Cat# CB101-01 E.coli BL21 (DE3) TIANGEN Cat# CB105 Biological samples Human postmortem spinal tissues; see details in Table S1 Central South University Xiangya School of Medicine and Nanjing Medical University N/A Chemicals, peptides, and recombinant proteins Penicillin-streptomycin Sangon Biotech Cat# E607011-0100 ROCK inhibitor STEMCELL Cat# Y-27632 DMEM/F12 Gibco Cat# C11330500BT DMEM Cytiva Cat# SH30022.01 FBS ExCell Bio Cat# FSD500 Essential 8TM medium Gibco Cat# A15169-01 Vitronectin (VTN) Gibco Cat# A14700 Cell dissociation reagent STEMCELL Cat# 07174 CD3 BD Cat# 55336 RPMI-1640 medium Gibco Cat# 11875093 Sodium pyruvate Gibco Cat# 11360070 Glutamax Gibco Cat# 35050061 Mitomycin C MCE Cat# HY-13316 JQ1 Sigma Cat# Sml1524 KOSR Thermo Cat# 10828028 NEAA Gibco Cat# 11140-050 bFGF Thermo Cat# PHG0263 Matrigel BD Cat# 356234 Puromycin Selleck Cat# S7417 Dispase Gibco Cat# 17105-041 N2 supplement Thermo Cat# 17502048 B27 Gibco Cat# 12587010 CHIR99021 Tocris Cat# 4423 DMH1 Tocris Cat# 4126 SB431542 Tocris Cat# 1614 Retinoic acid Sigma Cat# R2625 SAG Sigma Cat# 566660 Compound E MERCK Cat# 565790 cAMP Sigma Cat# A6885 Horse serum Absin Cat# abs989 Fluo-4 AM calcium indicator Thermo Cat# F10489 GenEscort Wisegen Cat# WIS1100 (Continued on next page) Neuron 114, 1–19.e1–e14, June 17, 2026 e2 .. REAGENT or RESOURCE SOURCE IDENTIFIER PEG8000 Sangon Biotech Cat# A600433-0500 Polybrene Solarbio Cat# H8761 Hygromycin Selleck Cat# S2908 CCCP MCE Cat# HY-100941 AP1903 MCE Cat# HY-16046 MTT labeling regent Roche Cat# 11465007001 Isoflurane RWD Cat# R510-22-10 Protease inhibitors NCM Biotech Cat# P001 Tris Sangon Biotech Cat# A501492-0005 NP-40 Sangon Biotech Cat# A100109-0100 Sodium deoxycholate Sangon Biotech Cat# A100613-001 SDS Sangon Biotech Cat# A500225-0001 β-mercaptoethanol Sigma Cat# M3148 Digitonin Sangon Biotech Cat# A601152-0100 Triton X-100 Sangon Biotech Cat# A600198 Glycine Sangon Biotech Cat# A502065-0005 Trifluoroacetic acid Sangon Biotech Cat# A501480 Acetonitrile Sangon Biotech Cat# A362610 Trypsin Promega Cat# V5113 TiO 2 columns Thermo Cat# A32993 Bovine serum albumin (BSA) MACKLIN Cat# A85022 Donkey serum (DS) VivaCell Cat# C2540-0100 IPTG Solarbio Cat# I8070 Imidazole Sigma Cat# 8142230005 EDC Cytiva Cat# BR100050 NHS Cytiva Cat# BR100050 HBS-EP buffer Cytiva Cat# BR100826 OCT SAKURA Cat# 4583 DNA ligase Sigma Cat# DUO92008 DNA polymerase Sigma Cat# DUO92008 Telmisartan Sigma Cat# PHR1855 MG132 MCE Cat# HY-13259 Eeyarestatin I (EerI) MCE Cat# HY-110078 Sendai Reprogramming Kit Thermo Cat# A16517 Phosphatase inhibitor complex I Sangon Biotech Cat# C500017 DAPI Solarbio Cat# C0060 PFA Sigma Cat# 8187151000 Sucrose Sigma Cat# V900116 Ethylene glycol Sigma Cat# 293237 H 2 O 2 Sigma Cat# H1009 α-Bungarotoxin(α-BTX), Cy3-conjugated Bosunlife Cat# B00018 EDTA Sangon Biotech Cat# A600107 Hoechst Solarbio Cat# C0030 MgCl 2 Sangon Biotech Cat# A601336-0500 HEPES Sangon Biotech Cat# A600264-0050 Avertin Sigma Cat# T48402 NaCl Sangon Biotech Cat# A501218 Thapsigargin drug (TG) MCE Cat# HY-13433 (Continued on next page) e3 Neuron 114, 1–19.e1–e14, June 17, 2026 .. REAGENT or RESOURCE SOURCE IDENTIFIER Ethanolamine-HCL Sigma Cat# E6133 Protease K TIANGEN Cat# x0317 Critical commercial assays DNeasy Blood & Tissue Kit TIANGEN Cat# DP304-03 TRIzolTM Reagent Sigma Cat# T9424 HiScript III RT SuperMix for qPCR Vazyme Cat# R323-01 2× SYBR Green qPCR Master Mix Vazyme Cat# Q312-02 BCA Protein Assay Kit Beyotime Biotechnology Cat# P0009 Protein A/G agarose beads Santa Cruz Cat# SC2003 Glutathione agarose beads ABclonal Cat# AS2044 Duolink Blocking Solution Sigma Cat# DUO92001 PLA probes Sigma Cat# DUO92005 HisTrap HP Cytiva Cat# 29051021 Malachite green reagent Sigma Cat# MAK307-1KT ECL chemiluminescence substrate Biosharp Cat# B2520B Ficoll-Paque PLUS Cytiva Cat# 17144002 CUT&Tag 4.0 High-Sensitivity Kit Novoprotein Cat# N259-YH01 Deposited data CRISPR data This study SRA: SRP601054 RNA-seq data This study SRA: SRP600123 RNA-seq and CUT&Tag data This study SRA: SRP659319 RNA sequencing data, sALS postmortem lumbar spinal Newton et al. 50 GEO: GSE255683 RNA sequencing data, sALS postmortem motor cortex Tam et al. 51 GEO: GSE122649 RNA sequencing data, C9-ALS postmortem front cortex Prudencio et al. 52 GEO: GSE67196 RNA sequencing data, sALS iMN Answer ALS database N/A RNA sequencing data, TDP-43(M337V) iMN Dafinca et al. 53 GEO: GSE147544 RNA sequencing data, C9-ALS iMN Catanese et al. 54 GEO: GSE168831 RNA sequencing data, FUS(P525L) iMN De Santis et al. 55 GEO: GSE94888 RNA sequencing data, VCP-ALS and TDP43-ALS iMN Ziff et al. 56 GEO: GSE214017 RNA sequencing data, FUS-KD, TAF15 KD and TDP-43 KD iMN Kapeli et al. 57 GEO: GSE77702 RNA sequencing data, C9-ALS iMN Sommer et al. 58 GEO: GSE201407 RNA sequencing data, FUS(H517Q) iMN Hawkins et al. 59 GEO: GSE203168 RNA sequencing data, C9-ALS iMN Abo-Rady et al. 60 GEO: GSE143743 RNA sequencing data, PFN1(G118V) mouse Barham et al. 61 GEO: GSE113924 RNA sequencing data, SOD1(G93A) mouse Herskovits et al. 62 GEO: GSE106803 RNA sequencing data, SOD1(G85R) mouse Henriques et al. 63 GEO: GSE106364 RNA sequencing data, SOD1(G85R) mouse Bandyopadhyay et al. 64 GEO: GSE38820 RNA sequencing data, SOD1(G93A) mouse Phatnani et al. 65 GEO: GSE43879 RNA sequencing data, SOD1(G93A) mouse Rudnick et al. 66 GEO: GSE100888 Experimental models: Cell lines SH-SY5Y ATCC ATCC Number: CRL-2266; RRID:CVCL_0019 (Continued on next page) Neuron 114, 1–19.e1–e14, June 17, 2026 e4
Labeling:Article Title: Targeting PGAM5-driven mitochondrial integrated stress response slows ALS progression across subtypes.
Article Snippet: REAGENT or RESOURCE SOURCE IDENTIFIER Mouse monoclonal anti-MAP2 Sigma Cat# M1406; RRID:AB_477171 Mouse monoclonal anti-HB9 DSHB Cat# 81.5C10; RRID:AB_2145209 Mouse monoclonal anti-ISL1 DSHB Cat# 40.2D6; RRID:AB_528315 Rabbit polyclonal anti-BIP HUABIO Cat# ER1706-50; RRID:AB_3069065 Rabbit polyclonal anti-ATP5B Proteintech Cat# 17247-1-AP; RRID:AB_2061878 Rabbit polyclonal anti-MFN1 Proteintech Cat# 13798-1-AP; RRID:AB_2266318 Rabbit Monoclonal anti-ATF4 CST Cat# 11815; RRID:AB_2616025 Goat Anti-Rabbit IgG H&L Novoprotein Cat# N269; RRID:AB_3697628 Goat polyclonal anti-OLIG2 Santa Cruz Cat# sc-19969; RRID:AB_2236477 Bacterial and virus strains E.coli DH5α TIANGEN Cat# CB101-01 E.coli BL21 (DE3) TIANGEN Cat# CB105 Biological samples Human postmortem spinal tissues; see details in Table S1 Central South University Xiangya School of Medicine and Nanjing Medical University N/A Chemicals, peptides, and recombinant proteins Penicillin-streptomycin Sangon Biotech Cat# E607011-0100 ROCK inhibitor STEMCELL Cat# Y-27632 DMEM/F12 Gibco Cat# C11330500BT DMEM Cytiva Cat# SH30022.01 FBS ExCell Bio Cat# FSD500 Essential 8TM medium Gibco Cat# A15169-01 Vitronectin (VTN) Gibco Cat# A14700 Cell dissociation reagent STEMCELL Cat# 07174 CD3 BD Cat# 55336 RPMI-1640 medium Gibco Cat# 11875093 Sodium pyruvate Gibco Cat# 11360070 Glutamax Gibco Cat# 35050061 Mitomycin C MCE Cat# HY-13316 JQ1 Sigma Cat# Sml1524 KOSR Thermo Cat# 10828028 NEAA Gibco Cat# 11140-050 bFGF Thermo Cat# PHG0263 Matrigel BD Cat# 356234 Puromycin Selleck Cat# S7417 Dispase Gibco Cat# 17105-041 N2 supplement Thermo Cat# 17502048 B27 Gibco Cat# 12587010 CHIR99021 Tocris Cat# 4423 DMH1 Tocris Cat# 4126 SB431542 Tocris Cat# 1614 Retinoic acid Sigma Cat# R2625 SAG Sigma Cat# 566660 Compound E MERCK Cat# 565790 cAMP Sigma Cat# A6885 Horse serum Absin Cat# abs989 Fluo-4 AM calcium indicator Thermo Cat# F10489 GenEscort Wisegen Cat# WIS1100 (Continued on next page) Neuron 114, 1–19.e1–e14, June 17, 2026 e2 .. REAGENT or RESOURCE SOURCE IDENTIFIER PEG8000 Sangon Biotech Cat# A600433-0500 Polybrene Solarbio Cat# H8761 Hygromycin Selleck Cat# S2908 CCCP MCE Cat# HY-100941 AP1903 MCE Cat# HY-16046 MTT labeling regent Roche Cat# 11465007001 Isoflurane RWD Cat# R510-22-10 Protease inhibitors NCM Biotech Cat# P001 Tris Sangon Biotech Cat# A501492-0005 NP-40 Sangon Biotech Cat# A100109-0100 Sodium deoxycholate Sangon Biotech Cat# A100613-001 SDS Sangon Biotech Cat# A500225-0001 β-mercaptoethanol Sigma Cat# M3148 Digitonin Sangon Biotech Cat# A601152-0100 Triton X-100 Sangon Biotech Cat# A600198 Glycine Sangon Biotech Cat# A502065-0005 Trifluoroacetic acid Sangon Biotech Cat# A501480 Acetonitrile Sangon Biotech Cat# A362610 Trypsin Promega Cat# V5113 TiO 2 columns Thermo Cat# A32993 Bovine serum albumin (BSA) MACKLIN Cat# A85022 Donkey serum (DS) VivaCell Cat# C2540-0100 IPTG Solarbio Cat# I8070 Imidazole Sigma Cat# 8142230005 EDC Cytiva Cat# BR100050 NHS Cytiva Cat# BR100050 HBS-EP buffer Cytiva Cat# BR100826 OCT SAKURA Cat# 4583 DNA ligase Sigma Cat# DUO92008 DNA polymerase Sigma Cat# DUO92008 Telmisartan Sigma Cat# PHR1855 MG132 MCE Cat# HY-13259 Eeyarestatin I (EerI) MCE Cat# HY-110078 Sendai Reprogramming Kit Thermo Cat# A16517 Phosphatase inhibitor complex I Sangon Biotech Cat# C500017 DAPI Solarbio Cat# C0060 PFA Sigma Cat# 8187151000 Sucrose Sigma Cat# V900116 Ethylene glycol Sigma Cat# 293237 H 2 O 2 Sigma Cat# H1009 α-Bungarotoxin(α-BTX), Cy3-conjugated Bosunlife Cat# B00018 EDTA Sangon Biotech Cat# A600107 Hoechst Solarbio Cat# C0030 MgCl 2 Sangon Biotech Cat# A601336-0500 HEPES Sangon Biotech Cat# A600264-0050 Avertin Sigma Cat# T48402 NaCl Sangon Biotech Cat# A501218 Thapsigargin drug (TG) MCE Cat# HY-13433 (Continued on next page) e3 Neuron 114, 1–19.e1–e14, June 17, 2026 .. REAGENT or RESOURCE SOURCE IDENTIFIER Ethanolamine-HCL Sigma Cat# E6133 Protease K TIANGEN Cat# x0317 Critical commercial assays DNeasy Blood & Tissue Kit TIANGEN Cat# DP304-03 TRIzolTM Reagent Sigma Cat# T9424 HiScript III RT SuperMix for qPCR Vazyme Cat# R323-01 2× SYBR Green qPCR Master Mix Vazyme Cat# Q312-02 BCA Protein Assay Kit Beyotime Biotechnology Cat# P0009 Protein A/G agarose beads Santa Cruz Cat# SC2003 Glutathione agarose beads ABclonal Cat# AS2044 Duolink Blocking Solution Sigma Cat# DUO92001 PLA probes Sigma Cat# DUO92005 HisTrap HP Cytiva Cat# 29051021 Malachite green reagent Sigma Cat# MAK307-1KT ECL chemiluminescence substrate Biosharp Cat# B2520B Ficoll-Paque PLUS Cytiva Cat# 17144002 CUT&Tag 4.0 High-Sensitivity Kit Novoprotein Cat# N259-YH01 Deposited data CRISPR data This study SRA: SRP601054 RNA-seq data This study SRA: SRP600123 RNA-seq and CUT&Tag data This study SRA: SRP659319 RNA sequencing data, sALS postmortem lumbar spinal Newton et al. 50 GEO: GSE255683 RNA sequencing data, sALS postmortem motor cortex Tam et al. 51 GEO: GSE122649 RNA sequencing data, C9-ALS postmortem front cortex Prudencio et al. 52 GEO: GSE67196 RNA sequencing data, sALS iMN Answer ALS database N/A RNA sequencing data, TDP-43(M337V) iMN Dafinca et al. 53 GEO: GSE147544 RNA sequencing data, C9-ALS iMN Catanese et al. 54 GEO: GSE168831 RNA sequencing data, FUS(P525L) iMN De Santis et al. 55 GEO: GSE94888 RNA sequencing data, VCP-ALS and TDP43-ALS iMN Ziff et al. 56 GEO: GSE214017 RNA sequencing data, FUS-KD, TAF15 KD and TDP-43 KD iMN Kapeli et al. 57 GEO: GSE77702 RNA sequencing data, C9-ALS iMN Sommer et al. 58 GEO: GSE201407 RNA sequencing data, FUS(H517Q) iMN Hawkins et al. 59 GEO: GSE203168 RNA sequencing data, C9-ALS iMN Abo-Rady et al. 60 GEO: GSE143743 RNA sequencing data, PFN1(G118V) mouse Barham et al. 61 GEO: GSE113924 RNA sequencing data, SOD1(G93A) mouse Herskovits et al. 62 GEO: GSE106803 RNA sequencing data, SOD1(G85R) mouse Henriques et al. 63 GEO: GSE106364 RNA sequencing data, SOD1(G85R) mouse Bandyopadhyay et al. 64 GEO: GSE38820 RNA sequencing data, SOD1(G93A) mouse Phatnani et al. 65 GEO: GSE43879 RNA sequencing data, SOD1(G93A) mouse Rudnick et al. 66 GEO: GSE100888 Experimental models: Cell lines SH-SY5Y ATCC ATCC Number: CRL-2266; RRID:CVCL_0019 (Continued on next page) Neuron 114, 1–19.e1–e14, June 17, 2026 e4
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