Key resources table.
| Reagent type (species) or resource |
Designation | Source or reference | Identifiers | Additional information |
|---|---|---|---|---|
| Cell line (Homo sapiens, male) | FP4 | This study | Available from the Anne Bang lab | |
| Genetic reagent (Homo sapiens, male) | FKRP C318Y | This study | TC1133 FKRP C318Y | Available from the Rita Perlingeiro lab |
| Genetic reagent (Homo sapiens, male) | FKRP KO | This study | H9 FKRP KO | Available from the Rita Perlingeiro lab |
| Cell line (Homo sapiens, male) | WT | PMID:22560081 | PLZ | Control line, available from the Rita Perlingeiro lab |
| Cell line (Homo sapiens, male) | Parental WT FKRP KO | WiCell | H9 | ESC control line (WA09) |
| Cell line (Homo sapiens, male) | Parental WT FKRP C318Y | PMID:26411904 | TC-1133 | Control line, available with RUCDR Infinite Biologics |
| Chemical compound, drug | CHIR99021 | Tocris | Cat# 4423 | 10 µM |
| Chemical compound, drug | LDN193189 | Cayman chemical | Cat# 19396 | 200 nM |
| Chemical compound, drug | SB431542 | Cayman chemical | Cat# 13031 | 10 µM |
| Chemical compound, drug | DAPT | Cayman chemical | Cat# 13197 | 10 µM |
| Chemical compound, drug | Dexamethasone | Cayman chemical | Cat# 11015 | 10 µM |
| Chemical compound, drug | Forskolin | Cayman chemical | Cat# 11018 | 10 µM |
| Chemical compound, drug | Ribitol | Sigma-Aldrich | Cat# A5502 | 50 mM |
| Chemical compound, drug | D-(−)-ribose | Sigma-Aldrich | Cat# R9629 | 10 mM |
| Chemical compound, drug | NAD+ | Sigma-Aldrich | Cat# N0632 | 100 µM |
| Chemical compound, drug | Sorbinil | Sigma-Aldrich | Cat# S7701 | 100 μM |
| Chemical compound, drug | Doxycycline | Sigma-Aldrich | Cat# D9891 | 1 µg/ml |
| Recombinant protein | Recombinant human FGF-basic | Peprotech | Cat# 100-18B | 5 ng/ml |
| Antibody | Anti-alpha dystroglycan (mouse monoclonal) | Millipore | Cat# 05–593, RRID:AB_309828 | Dilution 1:1000 (WB) |
| Antibody | Anti-alpha dystroglycan (mouse monoclonal) |
DSHB | Cat# IIH6 C4, RRID:AB_2617216 | Dilution 1:50 (IF) |
| Antibody | Anti-human dystroglycan (sheep polyclonal) | R and D Systems | Cat# AF6868, RRID:AB_10891298 | Dilution 1:1000 (WB) |
| Antibody | Anti-MHC (mouse monoclonal) | DSHB | Cat# MF20, RRID: AB_2147781 | Dilution 1:50 (IF) 1:200 (WB) |
| Antibody | Anti-laminin (rabbit polyclonal) | Sigma-Aldrich | Cat# L9393, RRID:AB_477163 | Dilution 1:1000 (WB) |
| Antibody | Anti-beta dystroglycan, concentrated (mouse monoclonal) | DSHB | Cat# MANDAG2 clone 7D11, RRID:AB_2211772 | Dilution 1:1500 (WB) |
| Antibody | Anti-OCT3/4 (mouse monoclonal) | SCBT | Cat# C-10, RRID: AB_628051 | Dilution 1:50 (IF) |
| Antibody | Anti-SOX2 (goat polyclonal) |
SCBT | Cat# Y-17, RRID: AB_2286684 | Dilution 1:50 (IF) |
| Antibody | Anti-NANOG (mouse monoclonal) | SCBT | Cat# H-2, RRID: AB_10918255 | Dilution 1:50 (IF) |
| Antibody | Anti-SSEA4 (mouse monoclonal) | SCBT | Cat# sc-21704, RRID: AB_628289 | Dilution 1:50 (IF) |
| Antibody | Alexa fluor 555 goat anti-mouse IgG and IgM (goat polyclonal) |
Thermo Fisher Scientific | Cat# A-21424, RRID: AB_141780
Cat# A-21042, RRID:AB_2535711 |
Dilution 1:500 (IF) |
| Antibody | DyLight 680 anti-rabbit IgG (goat polyclonal) |
Thermo Fisher Scientific | Cat# 35568, RRID:AB_614946 | Dilution 1:10000 (WB) |
| Antibody | DyLight 680 anti-mouse IgM (goat polyclonal) |
Thermo Fisher Scientific | Cat# SA5-10154, RRID:AB_2556734 | Dilution 1:10000 (WB) |
| Antibody | DyLight 680 anti-sheep IgG (rabbit polyclonal) |
Thermo Fisher Scientific | Cat# SA5-10058, RRID:AB_2556638 | Dilution 1:10000 (WB) |
| Antibody | DyLight 800 anti-mouse IgG (goat polyclonal) |
Thermo Fisher Scientific | Cat# SA5-10176, RRID:AB_2556756 | Dilution 1:10000 (WB) |
| Other | DAPI stain | SCBT | sc-3598 | (1.5 µg/ml) |
| Other | Laminin from Engelbreth-Holm-Swarm murine sarcoma basement membrane | Millipore Sigma | Cat# L2020 | 1 µg/µl |