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Sanbio Inc risdiplam 29028-1
(A) Schematic representation of the <t>Risdiplam</t> treatment. (B) Representative immunoblots for SMN in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from 3 technical replicates of one paediatric patient and one healthy donor. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (C) Representative immunoblots for COL1A1 in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from paediatric healthy donors, and paediatric and adult SMA patients. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (D) Quantification of SMN protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001. (E) Quantification of COL1A1 protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001.
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(A) Schematic representation of the <t>Risdiplam</t> treatment. (B) Representative immunoblots for SMN in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from 3 technical replicates of one paediatric patient and one healthy donor. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (C) Representative immunoblots for COL1A1 in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from paediatric healthy donors, and paediatric and adult SMA patients. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (D) Quantification of SMN protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001. (E) Quantification of COL1A1 protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001.
Filtered Circuit #29028 Xxx, supplied by Vyaire Medical Inc, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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(A) Schematic representation of the <t>Risdiplam</t> treatment. (B) Representative immunoblots for SMN in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from 3 technical replicates of one paediatric patient and one healthy donor. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (C) Representative immunoblots for COL1A1 in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from paediatric healthy donors, and paediatric and adult SMA patients. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (D) Quantification of SMN protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001. (E) Quantification of COL1A1 protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001.
Filtered Circuit (#29028 Xxx), supplied by Vyaire Medical Inc, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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ATCC 281 290282 malathion
(A) Schematic representation of the <t>Risdiplam</t> treatment. (B) Representative immunoblots for SMN in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from 3 technical replicates of one paediatric patient and one healthy donor. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (C) Representative immunoblots for COL1A1 in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from paediatric healthy donors, and paediatric and adult SMA patients. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (D) Quantification of SMN protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001. (E) Quantification of COL1A1 protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001.
281 290282 Malathion, supplied by ATCC, used in various techniques. Bioz Stars score: 94/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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ATCC bladder a549 80
(A) Schematic representation of the <t>Risdiplam</t> treatment. (B) Representative immunoblots for SMN in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from 3 technical replicates of one paediatric patient and one healthy donor. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (C) Representative immunoblots for COL1A1 in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from paediatric healthy donors, and paediatric and adult SMA patients. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (D) Quantification of SMN protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001. (E) Quantification of COL1A1 protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001.
Bladder A549 80, supplied by ATCC, used in various techniques. Bioz Stars score: 94/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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(A) Schematic representation of the Risdiplam treatment. (B) Representative immunoblots for SMN in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from 3 technical replicates of one paediatric patient and one healthy donor. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (C) Representative immunoblots for COL1A1 in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from paediatric healthy donors, and paediatric and adult SMA patients. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (D) Quantification of SMN protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001. (E) Quantification of COL1A1 protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001.

Journal: bioRxiv

Article Title: Translation-specific disruption of Col1a1 expression in multiple models of Spinal Muscular Atrophy can be rescued by Risdiplam

doi: 10.1101/2025.06.03.657600

Figure Lengend Snippet: (A) Schematic representation of the Risdiplam treatment. (B) Representative immunoblots for SMN in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from 3 technical replicates of one paediatric patient and one healthy donor. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (C) Representative immunoblots for COL1A1 in untreated (U) and Risdiplam-treated (R) patient-derived fibroblasts from paediatric healthy donors, and paediatric and adult SMA patients. The internal standard (Int. std.) is used to compare protein signal across different membranes. Each blot is accompanied by a corresponding total protein stain (lower panel). (D) Quantification of SMN protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001. (E) Quantification of COL1A1 protein level of Risdiplam-treated (R) relative to the untreated (U) fibroblasts. Protein levels were normalized for total protein stain and internal standard. Data are mean ± s.e.m. among n = 3 technical independent replicates. Significant changes were assessed using unpaired two-sided Student’s t-test, with Holm correction of p-values. * p-val<0.05, ** p-val<0.01, *** p-val<0.001, and **** p-val<0.0001.

Article Snippet: For Risdiplam (Sanbio, 29028-1) treatment, cells were treated at a final concentration of 0.5 μM and the treatment was repeated after 24 h. The total treatment time was 48 h.

Techniques: Western Blot, Derivative Assay, Staining