Journal: Stem cell research

Article Title: Generation of an induced pluripotent stem cell line (TRNDi030-A) from a patient with Farber disease carrying a homozygous p. Y36C (c. 107 A>G) mutation in ASAH1

doi: 10.1016/j.scr.2021.102387

Figure Lengend Snippet: Resource Table

Article Snippet: This study has established a human iPSC line (TRNDi030-A) from the fibroblasts of a male patient with Farber disease (GM20015, Coriell Institute) carrying a homozygous mutation, p. Y36C (c.107 A>G), in the second exon of ASAH1 ( , ).

Techniques: Mutagenesis

Journal: Toxicological Sciences

Article Title: Functional and Mechanistic Neurotoxicity Profiling Using Human iPSC-Derived Neural 3D Cultures

doi: 10.1093/toxsci/kfy218

Figure Lengend Snippet: (A) Human iPSC-derived 3D neural culture spheroids, approximately 600 μm diameter, imaged with ImageXpress Micro Confocal system, 20x magnification, transmitted light. (B) Fluorescent images were taken after staining cells with marker-specific antibodies as described in Materials and Methods section. Images were taken using 20× objective in confocal mode. Spheroids are composed of a co-culture of active cortical neurons (identified by MAP2; green) and astrocytes (identified by GFAP; red). (C) Staining for Aquaporin 4 (green) showed the presence of mature GFAP-positive astrocytes. (D) Staining for Synapsin I (red) showed the presence of mature MAP2-positive neurons. B, C, and D show composite projection images of 3D neural cultures (30 images, 15 μm apart). Nuclei are stained with Hoechst 33342 (blue).

Article Snippet: As a negative control we extracted RNA from the undifferentiated human iPSC line used to generate the 3D neural cultures (StemoniX).

Techniques: Derivative Assay, Staining, Marker, Co-Culture Assay

Journal: Toxicological Sciences

Article Title: Functional and Mechanistic Neurotoxicity Profiling Using Human iPSC-Derived Neural 3D Cultures

doi: 10.1093/toxsci/kfy218

Figure Lengend Snippet: (A) Gene expression profiling for neurotransmitters receptors. (B) Gene expression profiling for ion channels. Data presented for 3D neural cultures samples and adult brain samples. Both values were normalized to iPSC cells. KCNC1 and others represent Potassium Voltage-Gated Channels; SCN2A and other represent Sodium Voltage-Gated Channels; CACNA1D represent L-type calcium channels.

Article Snippet: As a negative control we extracted RNA from the undifferentiated human iPSC line used to generate the 3D neural cultures (StemoniX).

Techniques: Expressing

Journal: Stem cell research

Article Title: An induced pluripotent stem cell line (TRNDi009-C) from a Niemann-Pick disease type A patient carrying a heterozygous p.L302P (c.905 T > C) mutation in the SMPD1 gene

doi: 10.1016/j.scr.2019.101461

Figure Lengend Snippet: Characterization of TRNDi009-C iPSC line. A) Left: phase contrast imaging of TRNDi009-C colonies grown on Geltrex at passage 6. Right: Representative immunofluorescent images of iPSCs positive for stem cell markers: SOX2, OCT4, NANOG, and SSEA4. Nucleus is labelled with Hoechst 33342 (blue). B) Cytogenetic analysis showing a normal karyotype (46, XX). C) Detection of heterozygous mutation of a p.L302P (c.905 T > C) in exon 2 of the SMPD1 gene. D) Flow cytometry analysis of pluripotency protein markers: NANOG and TRA-1–60. E) RT-PCR verification for the clearance of the Sendai virus from reprogrammed cells. Sendai virus vector transduced fibroblasts were used as a positive control. F) Pathological analysis of teratoma from TRNDi009-C iPSC, showing a normal ectodermal, mesodermal, and endodermal differentiation.

Article Snippet: In this study, a human iPSC line was established from skin dermal fibroblasts of a 21-fetal-week-old female patient (GM03252, Coriell Institute) ( ).

Techniques: Imaging, Mutagenesis, Flow Cytometry, Reverse Transcription Polymerase Chain Reaction, Virus, Plasmid Preparation, Positive Control

Journal: Stem cell research

Article Title: An induced pluripotent stem cell line (TRNDi009-C) from a Niemann-Pick disease type A patient carrying a heterozygous p.L302P (c.905 T > C) mutation in the SMPD1 gene

doi: 10.1016/j.scr.2019.101461

Figure Lengend Snippet:

Article Snippet: In this study, a human iPSC line was established from skin dermal fibroblasts of a 21-fetal-week-old female patient (GM03252, Coriell Institute) ( ).

Techniques: Modification, Mutagenesis