hemizygous b6.sjl-tg sod1 g93a males  (Jackson Laboratory)

Journal: Non-coding RNA Research

Article Title: LncRNA levels in the central nervous system as novel potential players and biomarkers in amyotrophic lateral sclerosis

doi: 10.1016/j.ncrna.2025.05.017

Figure Lengend Snippet: Differentially expressed lncRNAs in the central nervous system of SOD1 G93A ALS mice. (A) Volcano plot of differentially expressed genes identified between SOD1 G93A mice and age-matched control group. Each point represents the fold-change of ALS mouse model at a given stage (pre-symptomatic, symptomatic or late symptomatic) and tissue (spinal cord, brainstem and frontal cortex). The blue dots denote down-regulated gene expression, the red dots denote up-regulated gene expression. (B – E) Venn diagrams of down- and up-regulated lncRNAs taking into account sex-mixed groups of ALS and WT mice (B down-regulated, C up-regulated) and separating by sex (D down-regulated, E up-regulated). Yellow, green and purple colours correspond to the spinal cord, brainstem and frontal cortex, respectively. N = 24 (spinal cord) and N = 20 (brainstem and frontal cortex) per stage, balanced in sex and genotype. Results shown were obtained by RT-qPCR; supplemental information for this figure can be found in and .

Article Snippet: WT and transgenic SOD1 G93A mice were obtained by crossbreeding hemizygous B6.SJL-Tg SOD1 G93A males from The Jackson Laboratory (Bar Harbor, ME, USA) with B6.SJL females from Janvier Labs (Saint-Berthevin Cedex, France).

Techniques: Control, Gene Expression, Quantitative RT-PCR

Journal: Non-coding RNA Research

Article Title: LncRNA levels in the central nervous system as novel potential players and biomarkers in amyotrophic lateral sclerosis

doi: 10.1016/j.ncrna.2025.05.017

Figure Lengend Snippet: LncRNA expression pattern in the central nervous system of SOD1 G93A ALS mice. Heat map of lncRNA fold change of male (A) and female (B) SOD1 G93A mice in different regions of the central nervous system. LncRNA fold change is expressed as a value rated from 0 to >3, calculated as the ratio of RNA expression levels by RT-qPCR in ALS mice compared to their age-matched WT littermates. Xist expression is absent in males, as its transcription is restricted to individuals with two X chromosomes. N = 24 (spinal cord) and N = 20 (brainstem and frontal cortex) per stage, balanced in sex and genotype. Supplemental information for this figure can be found in . PS: presymptomatic stage (P60), ES: symptomatic stage (P90-P100), LS: late symptomatic stage (P120). ∗p < 0.05, ∗∗p < 0.01, ∗∗∗p < 0.001.

Article Snippet: WT and transgenic SOD1 G93A mice were obtained by crossbreeding hemizygous B6.SJL-Tg SOD1 G93A males from The Jackson Laboratory (Bar Harbor, ME, USA) with B6.SJL females from Janvier Labs (Saint-Berthevin Cedex, France).

Techniques: Expressing, RNA Expression, Quantitative RT-PCR

Journal: Non-coding RNA Research

Article Title: LncRNA levels in the central nervous system as novel potential players and biomarkers in amyotrophic lateral sclerosis

doi: 10.1016/j.ncrna.2025.05.017

Figure Lengend Snippet: LncRNAs Gas5 and Myoparr as potential modifiers of ALS progression in SOD1 G93A mice. (A,B) Gas5 levels in the spinal cord of females at the humane endpoint correlate with muscle strength failure onset (A) and disease duration (B). (C,D) Gas5 levels in the frontal cortex of terminal SOD1 G93A males correlates with motor coordination and balance failure onset (C) and disease duration (D). (E) Gas5 levels in the brainstem at the humane endpoint correlate with SOD1 G93A mice survival. (F) Myoparr levels in the frontal cortex of terminal SOD1 G93A males correlates with survival. For this experiment, N = 23 SOD1 G93A mice (n = 11 males, n = 12 females) at the humane endpoint. Results were obtained by RT-qPCR.

Article Snippet: WT and transgenic SOD1 G93A mice were obtained by crossbreeding hemizygous B6.SJL-Tg SOD1 G93A males from The Jackson Laboratory (Bar Harbor, ME, USA) with B6.SJL females from Janvier Labs (Saint-Berthevin Cedex, France).

Techniques: Quantitative RT-PCR

Journal: Frontiers in Neuroscience

Article Title: Targeting oxidized phosphatidylcholines in SOD1-associated ALS: therapeutic potential of PC-OxPL-VecTab ®

doi: 10.3389/fnins.2025.1620181

Figure Lengend Snippet: (A) Left panel: PC-OxPL expression in SOD1 G93A motor neurons (% of PC-OxPL+ cells). Values are expressed as means (wt = 1.000; SOD1 G93A = 1.239) ± SD. Unpaired two-tailed t -test; ** p = 0.0024. Three independent experiments, n = 2–4 replicates each. Right panel: Representative images show increased % of PC-OxPL+ cells in SOD1 G93A in comparison to wt motor neurons. Scale bar = 20 μM (PC-OxPL, Cy5). (B) Venn diagram depicting overlapping DE transcripts between SOD1 G93A and wt motor neurons exposed to PC-OxPL (PONPC). Both NanoString neuropathology and neuroinflammation gene expression panels were considered in the analysis. (C) Functional enrichment analysis of transcriptomic changes following AAV5.2-PC-OxPL-VecTab ® transduction of SOD1 G93A motor neurons. Only the five most enriched terms of each category are graphically represented (GO, Gene Ontology; padj, adjusted p -value; MF, molecular function; BP, biological process; CC, cellular component). (D) Heatmap representation of SOD1 G93A transcriptome normalization following AAV5.2-PC-OxPL-VecTab ®. Cell values represent the mean FC in relation to wt levels (FC, fold-change). (E) Axonal pathology in SOD1 G93A motor neurons exposed to PC-OxPL is mitigated by PC-OxPL-VecTab®. Upper panel: Values are expressed as means ± SD and normalized to the 25 μM PSPC condition. The number of axons was counted on the distal compartment as per TuJ-1 detection, used as a neuronal marker; n = 2 independent experiments. Lower panel: Representative panel of the distal compartment depicting PC-OxPL toxicity and neutralization by PC-OxPL-VecTab ® in SOD1 G93A motor neurons.

Article Snippet: Transgenic SOD1 G93A mice ( ) were acquired from Jackson Laboratory (JAX Stock #002726, hybrid C57BL/6 and SJL background).

Techniques: Expressing, Two Tailed Test, Comparison, Gene Expression, Functional Assay, Transduction, Marker, Neutralization

Journal: Frontiers in Neuroscience

Article Title: Targeting oxidized phosphatidylcholines in SOD1-associated ALS: therapeutic potential of PC-OxPL-VecTab ®

doi: 10.3389/fnins.2025.1620181

Figure Lengend Snippet: (A) Study design and treatment groups in wt and SOD1 G93A mice. (B) Vector DNA levels (gc/μg DNA) in cervical and lumbar spinal cord, brain cortex, and liver in PC-OxPL-VecTab ® -treated SOD1 G93A at day 70 of age (3 weeks post-injection). vDNA levels were determined by qPCR analysis and quantified based on a plasmid standard curve. Columns represent mean ± SD. (C) PC-OxPL-VecTab ® mRNA expression levels in cervical and lumbar spinal cord, brain cortex, and liver in PC-OxPL-VecTab ® -treated SOD1 G93A at day 70 of age (3 weeks post-injection). Transgene mRNA levels were measured by RT-qPCR and quantified as FC to the housekeeping (HKG) gene mHPRT1. Columns represent mean ± SD. One of the animals with low levels of vDNA (< 3×10 3 gc/μg DNA) showed undetectable levels of PC-OxPL-VecTab ® mRNA. (D) Plasma PC-OxPL concentrations (in ng/mL) determined in wt and SOD1 G93A mice over time (killed at days 45, 70, and 90 of age). Values are expressed as means ± SD. Unpaired t-test with Welch correction; **** p < 0.0001; *** p < 0.001; ** p < 0.01; * p < 0.05, n = 4–6 depending on lipid and time point. (E) Log 2 (FC) converted the effect of PC-OxPL-VecTab ® -treated SOD1 G93A mice vs. vehicle-SOD1 G93A mice for the 19 detected PC-OxPL species. A negative value represents a decrease in a specific PC-OxPL upon treatment with AAV5.2-PC-OxPL-VecTab ® . Two-way ANOVA; **** p < 0.0001; *** p < 0.001; ** p < 0.01; * p < 0.05. n = 9–12, depending on lipid and treatment.

Article Snippet: Transgenic SOD1 G93A mice ( ) were acquired from Jackson Laboratory (JAX Stock #002726, hybrid C57BL/6 and SJL background).

Techniques: Plasmid Preparation, Injection, Expressing, Quantitative RT-PCR, Clinical Proteomics