Appendix 1—key resources table.
| Reagent type (species) or resource | Designation | Source or reference | Identifiers | Additional information |
|---|---|---|---|---|
| Cell line (Homo-sapiens) | HEK293T | ATCC | Cat#: CRL-3216 | |
| Cell line (Homo-sapiens) | HEK293T | Abgent | Cat#: CL1032 | |
| Cell line (Homo-sapiens) | Fibroblasts harbouring L444P β-glucocerebrosidase | Coriell Institute | Cat#: GM20272 | |
| Transfected construct (Human) | siRNA to SERPINH1 | Dharmacon | Cat#: J-011230-05-0005 | |
| Transfected construct (Human) | siRNA to SERPINH1 | Dharmacon | Cat#: J-011230-06-0005 | |
| Transfected construct (Human) | siRNA, non-targeting control | Dharmacon | Cat#: D-001810-01-20 | |
| Biological sample (rat) | E18 hippocampus | BrainBits | Cat#: SDEHP | |
| Biological sample (rat) | E18 cortex | BrainBits | Cat#: SDECX | |
| Antibody | anti-GABAAα1 (mouse monoclonal) | Millipore | Cat#: MAB339 | WB (1:2000) |
| Antibody | anti-GABAAβ2/3 (mouse monoclonal) | Millipore | Cat#: 05–474 | WB (1:1000) IF (1:250) |
| Antibody | anti-GABAAβ2 (rabbit polyclonal) | Millipore | Cat#: AB5561 | WB (1:1000) |
| Antibody | anti-GABAAγ2 (rabbit polyclonal) | Millipore | Cat#: AB5559 | WB (1:1000) |
| Antibody | anti-NeuN (rabbit polyclonal) | Millipore | Cat#: ABN78 | IF (1:500) |
| Antibody | anti-GABAAα1 (rabbit polyclonal) | R&D systems | Cat#: PPS022 | WB (1:1000) |
| Antibody | anti-GABAAα1 (goat polyclonal) | Santa Cruz Biotechnology | Cat#: SC-31405 | WB (1:1000) |
| Antibody | anti-nAChR α4 (mouse monoclonal) | Santa Cruz Biotechnology | Cat#: sc-74519 | WB (1:1000) |
| Antibody | anti-GABAAα1 (rabbit polyclonal) | Synaptic Systems | Cat#: 224203 | IF (1:250) |
| Antibody | anti-GABAAγ2 (rabbit polyclonal) | Synaptic Systems | Cat#: 224003 | IF (1:250) |
| Antibody | anti-nAChR β2 (rabbit polyclonal) | Proteintech | Cat#: 17844–1-AP | WB (1:1000) |
| Antibody | anti-nAChR α7 (rabbit polyclonal) | Proteintech | Cat#: 21379–1-AP | WB (1:1000) |
| Antibody | anti-ATF6 (rabbit polyclonal) | Proteintech | Cat#: 24169–1-AP | WB (1:2000) |
| Antibody | anti-FLAG (rabbit polyclonal) | Proteintech | Cat#: 20543–1-AP | WB (1:2000) |
| Antibody | anti-Hsp47 (mouse monoclonal) | Proteintech | Cat#: 67863–1-lg | WB (1:1000) |
| Antibody | anti-5-HT3A (goat polyclonal) | Origene | Cat#: TA302602 | WB (1:1000) |
| Antibody | anti-GluN2A (rabbit monoclonal) | Abcam | Cat#: ab124913 | WB (1:3000) |
| Antibody | anti-Hsp47 (rabbit monoclonal) | Abcam | Cat#: ab109117 | WB (1:1000) IF: (1:250) |
| Antibody | anti-Grp78 (rabbit monoclonal) | Abcam | Cat#: ab108613 | WB (1:2000) |
| Antibody | anti-Na+/K+ATPase (rabbit monoclonal) | Abcam | Cat#: ab76020 | WB (1:10,000) |
| Antibody | anti-hERG (rabbit polyclonal) | ThermoFisher | Cat#: PA3-860 | WB (1:10,000) |
| Antibody | anti-XBP1s (rabbit monoclonal) | Cell Signaling | Cat#: 12782 S | WB (1:1000) |
| Antibody | anti-CHOP (mouse monoclonal) | Cell Signaling | Cat#: 2895 S | WB (1:1000) |
| Antibody | anti-His (mouse monoclonal) | Cell Signaling | Cat#: 2366 S | WB (1:1000) |
| Antibody | anti- glucocerebrosidase (rabbit polyclonal) | Sigma | Cat#: G4046 | WB (1:1000) |
| Antibody | anti-FLAG (mouse monoclonal) | Sigma | Cat#: F1804 | WB (1:2000) |
| Antibody | anti-β-actin (mouse monoclonal) | Sigma | Cat#: A1978 | WB (1:20,000) |
| Antibody | Fluorescent anti-β-actin Rhodamine | Biorad | Cat#: 12004163 | WB (1:10,000) |
| Antibody | anti- Grp78 (rabbit polyclonal) | Abgent | Cat#: AP5041c | WB (1:5000) |
| Antibody | anti-Hsp47 (mouse monoclonal) | Enzo Life Sciences | Cat#: ADI-SPA-470-F | WB (1:2000) IF (1:250) |
| Antibody | Alexa 594-conjugated goat anti-rabbit | ThermoFisher | Cat#: A11037 | IF (1:500) |
| Antibody | Alexa 594-conjugated goat anti-mouse | ThermoFisher | Cat#: A11032 | IF (1:500) |
| Antibody | Alexa 405-conjugated goat anti-rabbit | ThermoFisher | Cat#: A31556 | IF (1:500) |
| Recombinant DNA reagent | pCMV6-GABRA1-CFP (plasmid) | This paper | See Materials and Methods, Section Plasmids and siRNAs | |
| Recombinant DNA reagent | pCMV6-GABRB2 (plasmid) | Origene | Cat#: RC216424 | |
| Recombinant DNA reagent | pCMV6-GABRB2-YFP (plasmid) | This paper | See Materials and Methods, Section Plasmids and siRNAs | |
| Recombinant DNA reagent | pCMV6-GABRG2 (plasmid) | Origene | Cat#: RC209260 | |
| Recombinant DNA reagent | pCMV6-HTR3A (plasmid) | Origene | Cat#: SC122578 | |
| Recombinant DNA reagent | pCMV6-HTR3B (plasmid) | Origene | Cat#: MR206966 | |
| Recombinant DNA reagent | pCMV6-KCNH2 (plasmid) | Origene | Cat#: RC215928 | |
| Recombinant DNA reagent | pCMV6-Entry Vector (plasmid) | Origene | Cat#: PS100001 | |
| Recombinant DNA reagent | pcDNA3.1-GRIN2A (plasmid) | GenScript | Cat#: OHu24642D | |
| Recombinant DNA reagent | pcDNA3.1-GRIN1 (plasmid) | GenScript | Cat#: OHu22255D | |
| Recombinant DNA reagent | CHRNA4 (plasmid) | Addgene | RRID: Addgene_24271 | |
| Recombinant DNA reagent | CHRNA4-YFP (plasmid) | Addgene | RRID: Addgene_15245 | |
| Recombinant DNA reagent | CHRNB2 (plasmid) | Addgene | RRID: Addgene_24272 | |
| Recombinant DNA reagent | CHRNB2-CFP (plasmid) | Addgene | RRID: Addgene_15106 | |
| Recombinant DNA reagent | pcDNA3.1-CHRNA7 (plasmid) | Addgene | RRID: Addgene_62276 | |
| Recombinant DNA reagent | pcDNA3.1-CHRNA7-cerulean (plasmid) | https://pubmed.ncbi.nlm.nih.gov/23586521/ | ||
| Recombinant DNA reagent | pcDNA3.1-CHRNA7-venus (plasmid) |
https://pubmed.ncbi.nlm.nih.gov/23586521/ | ||
| Recombinant DNA reagent | pCMV6-XL5-SERPINH1 (plasmid) | Origene | Cat#: SC119367 | |
| Recombinant DNA reagent | pRK5-HA-Ubiquitin (plasmid) | Addgene | RRID: Addgene_17608 | |
| Recombinant DNA reagent | psPAX2 (plasmid) | Addgene | RRID: Addgene_12260 | |
| Recombinant DNA reagent | pMD2.G (plasmid) | Addgene | RRID: Addgene_12259 | |
| Recombinant DNA reagent | pCIG3 (plasmid) | Addgene | RRID: Addgene_78264 | |
| Recombinant DNA reagent | pHRIG-AktDN (plasmid) | Addgene | RRID: Addgene_53597 | |
| Recombinant DNA reagent | pHRIG-SERPINH1 (plasmid) | This paper | See Materials and Methods, Section Plasmids and siRNAs | |
| Recombinant DNA reagent | Scrambled siRNA GFP lentivector (plasmid) | Applied Biological Materials | Cat#: LV015-G | |
| Recombinant DNA reagent | SERPINH1 siRNA lentivector (smartpool) (plasmid) | Applied Biological Materials | Cat#: 435050960395 | |
| Peptide, recombinant protein | GST | Novus | Cat#: NBC1-18537 | |
| Peptide, recombinant protein | GST-GABRA1 | Abnova | Cat#: H00002554-P01 | |
| Peptide, recombinant protein | GST-GABRB2 | Abnova | Cat#: H00002561-P01 | |
| Peptide, recombinant protein | His-Hsp47 | Novus | Cat#: NBC1-22576 | |
| Peptide, recombinant protein | FLAG-ZIP7 | Origene | Cat#: TP313722 | |
| Peptide, recombinant protein | hERG | Abnova | Cat#: H00003757-G01 | |
| Peptide, recombinant protein | His-GABRA1-ERD | MyBioSource | Cat#: MBS948971 | |
| Peptide, recombinant protein | His-GABRB2-ERD | MyBioSource | Cat#: MBS953526 | |
| Peptide, recombinant protein | Hsp47 | Abcam | Cat#: ab86918 | |
| Commercial assay or kit | GenBuilder cloning kit | GenScript | Cat#: L00701 | |
| Chemical compound, drug | AA147 | Tocris Bioscience | Cat#: 6759 | |
| Chemical compound, drug | AA263 | Sigma | Cat#: R699470 | |
| Chemical compound, drug | DAPI | ThermoFisher | Cat#: D1306 | |
| Software, algorithm | ImageJ | National Institutes of Health | https://imagej.nih.gov/ij/ | |
| Software, algorithm | pClamp10 | Molecular Devices | https://www.moleculardevices.com/products/axon-patch-clamp-system | |
| Software, algorithm | Automatic patch clamping | Ionflux Mercury16 | https://www.fluxionbio.com/ionflux-mercury-automated-patch-clamp | |
| Software, algorithm | JASCO Spectra Manager | JASCO | ||
| Software, algorithm | Monolith NT.115 | NanoTemper | ||
| Software, algorithm | Origin | Origin Lab | https://www.originlab.com/ | |
| Software, algorithm | PyMOL | Schrodinger | https://pymol.org/ |