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(A) Timeline of disease progression of MATR3 S85C KI mice ( Matr3 S85C/S85C ). (B) Representative images of Calbindin-positive Purkinje cells in the whole cerebellum of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 20, and 30 weeks of age. Scale bars indicate 500 μm. (C) Quantification of the number of Purkinje cell soma throughout the whole cerebellum at 10, 20, and 30 weeks of age. Each dot represents the average of 4 sections of a single animal; N=3-6 mice per genotype. (D) Representative images of the neuromuscular junctions (NMJs) at 10 and 30 weeks of age. The presynaptic terminal (stained using Neurofilament H, synapsin, and <t>synaptophysin)</t> is shown in green and the motor endplate (α-bungarotoxin-positive staining which targets acetylcholine receptor) in red. Denervated NMJs are indicated by a white asterisk. (E) Quantification of partial denervation at the NMJs. Denervation was quantified by partial overlap (>1%, <80%) between the red and green channels. N=5 mice per genotype. (F) Quantification of axonal blebbing, which was determined by the presence of thick, rotund staining at the presynaptic terminal. N=5 mice per genotype. (G) Quantification of the motor endplate area. A single dot represents the average of >100 NMJs of a single animal; N=5 mice per genotype. (H-I) Relative expression (ΔΔC t analysis) of Chrna1 and Chrng from total RNA extracted from tibialis anterior (TA) muscle of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 30, and 60 weeks of age as measured by quantitative RT-PCR (N=8-12, male and female mice combined, bar heights depict mean ± SEM with each dot representing a single animal, significance determined by multiple unpaired t-test).
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(A) Timeline of disease progression of MATR3 S85C KI mice ( Matr3 S85C/S85C ). (B) Representative images of Calbindin-positive Purkinje cells in the whole cerebellum of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 20, and 30 weeks of age. Scale bars indicate 500 μm. (C) Quantification of the number of Purkinje cell soma throughout the whole cerebellum at 10, 20, and 30 weeks of age. Each dot represents the average of 4 sections of a single animal; N=3-6 mice per genotype. (D) Representative images of the neuromuscular junctions (NMJs) at 10 and 30 weeks of age. The presynaptic terminal (stained using Neurofilament H, synapsin, and <t>synaptophysin)</t> is shown in green and the motor endplate (α-bungarotoxin-positive staining which targets acetylcholine receptor) in red. Denervated NMJs are indicated by a white asterisk. (E) Quantification of partial denervation at the NMJs. Denervation was quantified by partial overlap (>1%, <80%) between the red and green channels. N=5 mice per genotype. (F) Quantification of axonal blebbing, which was determined by the presence of thick, rotund staining at the presynaptic terminal. N=5 mice per genotype. (G) Quantification of the motor endplate area. A single dot represents the average of >100 NMJs of a single animal; N=5 mice per genotype. (H-I) Relative expression (ΔΔC t analysis) of Chrna1 and Chrng from total RNA extracted from tibialis anterior (TA) muscle of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 30, and 60 weeks of age as measured by quantitative RT-PCR (N=8-12, male and female mice combined, bar heights depict mean ± SEM with each dot representing a single animal, significance determined by multiple unpaired t-test).
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(A) Timeline of disease progression of MATR3 S85C KI mice ( Matr3 S85C/S85C ). (B) Representative images of Calbindin-positive Purkinje cells in the whole cerebellum of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 20, and 30 weeks of age. Scale bars indicate 500 μm. (C) Quantification of the number of Purkinje cell soma throughout the whole cerebellum at 10, 20, and 30 weeks of age. Each dot represents the average of 4 sections of a single animal; N=3-6 mice per genotype. (D) Representative images of the neuromuscular junctions (NMJs) at 10 and 30 weeks of age. The presynaptic terminal (stained using Neurofilament H, synapsin, and <t>synaptophysin)</t> is shown in green and the motor endplate (α-bungarotoxin-positive staining which targets acetylcholine receptor) in red. Denervated NMJs are indicated by a white asterisk. (E) Quantification of partial denervation at the NMJs. Denervation was quantified by partial overlap (>1%, <80%) between the red and green channels. N=5 mice per genotype. (F) Quantification of axonal blebbing, which was determined by the presence of thick, rotund staining at the presynaptic terminal. N=5 mice per genotype. (G) Quantification of the motor endplate area. A single dot represents the average of >100 NMJs of a single animal; N=5 mice per genotype. (H-I) Relative expression (ΔΔC t analysis) of Chrna1 and Chrng from total RNA extracted from tibialis anterior (TA) muscle of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 30, and 60 weeks of age as measured by quantitative RT-PCR (N=8-12, male and female mice combined, bar heights depict mean ± SEM with each dot representing a single animal, significance determined by multiple unpaired t-test).
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Image Search Results


Journal: iScience

Article Title: TXNRD1-driven enteric glial remodeling promotes neuronal dysfunction in Hirschsprung disease

doi: 10.1016/j.isci.2026.117636

Figure Lengend Snippet:

Article Snippet: Mouse anti-Synaptophysin (SYN) , Santa Cruz Biotechnology , Cat# sc-365488.

Techniques: Model Organisms, Recombinant, CCK-8 Assay, Apoptosis Assay, ROS Assay, Calcium Assay, Enzyme-linked Immunosorbent Assay, Generated, Software, Genotyping

(A) Timeline of disease progression of MATR3 S85C KI mice ( Matr3 S85C/S85C ). (B) Representative images of Calbindin-positive Purkinje cells in the whole cerebellum of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 20, and 30 weeks of age. Scale bars indicate 500 μm. (C) Quantification of the number of Purkinje cell soma throughout the whole cerebellum at 10, 20, and 30 weeks of age. Each dot represents the average of 4 sections of a single animal; N=3-6 mice per genotype. (D) Representative images of the neuromuscular junctions (NMJs) at 10 and 30 weeks of age. The presynaptic terminal (stained using Neurofilament H, synapsin, and synaptophysin) is shown in green and the motor endplate (α-bungarotoxin-positive staining which targets acetylcholine receptor) in red. Denervated NMJs are indicated by a white asterisk. (E) Quantification of partial denervation at the NMJs. Denervation was quantified by partial overlap (>1%, <80%) between the red and green channels. N=5 mice per genotype. (F) Quantification of axonal blebbing, which was determined by the presence of thick, rotund staining at the presynaptic terminal. N=5 mice per genotype. (G) Quantification of the motor endplate area. A single dot represents the average of >100 NMJs of a single animal; N=5 mice per genotype. (H-I) Relative expression (ΔΔC t analysis) of Chrna1 and Chrng from total RNA extracted from tibialis anterior (TA) muscle of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 30, and 60 weeks of age as measured by quantitative RT-PCR (N=8-12, male and female mice combined, bar heights depict mean ± SEM with each dot representing a single animal, significance determined by multiple unpaired t-test).

Journal: bioRxiv

Article Title: Early MATR3 loss and distinct neurodegenerative molecular signatures precede the onset of neuropathology in motor neurons and Purkinje cells of MATR3 S85C knock-in mouse model of ALS

doi: 10.64898/2026.08.26.747343

Figure Lengend Snippet: (A) Timeline of disease progression of MATR3 S85C KI mice ( Matr3 S85C/S85C ). (B) Representative images of Calbindin-positive Purkinje cells in the whole cerebellum of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 20, and 30 weeks of age. Scale bars indicate 500 μm. (C) Quantification of the number of Purkinje cell soma throughout the whole cerebellum at 10, 20, and 30 weeks of age. Each dot represents the average of 4 sections of a single animal; N=3-6 mice per genotype. (D) Representative images of the neuromuscular junctions (NMJs) at 10 and 30 weeks of age. The presynaptic terminal (stained using Neurofilament H, synapsin, and synaptophysin) is shown in green and the motor endplate (α-bungarotoxin-positive staining which targets acetylcholine receptor) in red. Denervated NMJs are indicated by a white asterisk. (E) Quantification of partial denervation at the NMJs. Denervation was quantified by partial overlap (>1%, <80%) between the red and green channels. N=5 mice per genotype. (F) Quantification of axonal blebbing, which was determined by the presence of thick, rotund staining at the presynaptic terminal. N=5 mice per genotype. (G) Quantification of the motor endplate area. A single dot represents the average of >100 NMJs of a single animal; N=5 mice per genotype. (H-I) Relative expression (ΔΔC t analysis) of Chrna1 and Chrng from total RNA extracted from tibialis anterior (TA) muscle of Matr3 +/+ and Matr3 S85C/S85C mice at 10, 30, and 60 weeks of age as measured by quantitative RT-PCR (N=8-12, male and female mice combined, bar heights depict mean ± SEM with each dot representing a single animal, significance determined by multiple unpaired t-test).

Article Snippet: The following primary antibodies were used: synaptophysin antibody (rabbit polyclonal, Synaptic Systems 101 002, 1:200), synapsin I antibody (rabbit polyclonal, ab64581, 1:200), and neurofilament H antibody (chicken polyclonal, ab4680, 1:200).

Techniques: Biomarker Discovery, Staining, Expressing, Quantitative RT-PCR