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fast twitch myhc type iix (Developmental Studies Hybridoma Bank)

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Developmental Studies Hybridoma Bank fast twitch myhc type iix
Fast Twitch Myhc Type Iix, supplied by Developmental Studies Hybridoma Bank, used in various techniques. Bioz Stars score: 99/100, based on 5324 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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Cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries, together with a cocktail of mouse monoclonal antibodies against MyHC type I (BA-D5c, MIgG2b. 1:100), MyHC type IIA (SC-71c, MIgG1, 1:100), and MyHC type IIB (BF-F3c, MIgM, 1:100). Primary antibody staining was visualized using Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 350 (to detect laminin), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect SC-71), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), and Goat Anti-Mouse IgM Alexa Fluor 647 (to detect BF-F3). Laminin, BA-D5, SC-71, and BF-F3 staining were pseudo colored white, red, green, and blue respectively. Scale bars 100 µm.

Journal: bioRxiv

Article Title: Rapid High-Throughput Analysis of Bovine Skeletal Muscle Fiber Morphology via Automated Fluorescent Microscopy and MuscleBos software

doi: 10.64898/2025.12.18.695234

Figure Lengend Snippet: Cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries, together with a cocktail of mouse monoclonal antibodies against MyHC type I (BA-D5c, MIgG2b. 1:100), MyHC type IIA (SC-71c, MIgG1, 1:100), and MyHC type IIB (BF-F3c, MIgM, 1:100). Primary antibody staining was visualized using Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 350 (to detect laminin), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect SC-71), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), and Goat Anti-Mouse IgM Alexa Fluor 647 (to detect BF-F3). Laminin, BA-D5, SC-71, and BF-F3 staining were pseudo colored white, red, green, and blue respectively. Scale bars 100 µm.

Article Snippet: Primary antibodies tested included those against dystrophin [Developmental Studies Hybridoma Bank (DSHB), MANDYS1(3B7)s, MIgG2a, 1:10], Laminin 1 + 2 (Abcam, ab7463, Rabbit, 1:100), MyHC type I (DSHB, BA-D5c, MIgG2b, 1:100), MyHC type IIA (DSHB, SC-71c, MIgG1, 1:100), MyHC type IIX (DSHB, 6H1s, MIgM, 1:10), and all but type MyHC IIX (DSHB, BF-35c, MIgG1, 1:100).

Techniques: Staining, Bioprocessing

A: Cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against dystrophin [MANDYS1(3B7)s, MIgG2a, 1:10] to label the label the myofiber boundaries, together with a cocktail of mouse monoclonal primary antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100) and MyHC type IIA (SC-71c, MIgG1, 1:100). Primary antibody staining was visualized using Alexa Fluor-conjugated secondary antibodies (1: 500) including Goat Anti-Mouse IgG2a Alexa Fluor 647 (to detect dystrophin), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect SC-71), and Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5). Dystrophin, BA-D5, and SC-71 staining was pseudo colored white, red, and green, respectively. B: Serial cross-sections from the same bovine longissimus dorsi muscle biopsy sample shown in panel A were stained with a primary antibody against dystrophin [MANDYS1(3B7)s, MIgG2a, 1:10] to label the label the myofiber boundaries in combination with a cocktail of primary antibodies against MyHC type IIX (6H1s, MIgM, 1:10) and all MyHC isoforms except for type IIX (BF-35c, MIgG1, 1:100). Primary antibody staining was visualized using Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Mouse IgG2a Alexa Fluor 647 (to detect dystrophin), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35), and Goat Anti-Mouse IgM Alexa Fluor 555 (to detect 6H1). Dystrophin, BF-35, and 6H1 staining were pseudo colored white, red, and green, respectively. Scale bars are 200 µm.

Journal: bioRxiv

Article Title: Rapid High-Throughput Analysis of Bovine Skeletal Muscle Fiber Morphology via Automated Fluorescent Microscopy and MuscleBos software

doi: 10.64898/2025.12.18.695234

Figure Lengend Snippet: A: Cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against dystrophin [MANDYS1(3B7)s, MIgG2a, 1:10] to label the label the myofiber boundaries, together with a cocktail of mouse monoclonal primary antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100) and MyHC type IIA (SC-71c, MIgG1, 1:100). Primary antibody staining was visualized using Alexa Fluor-conjugated secondary antibodies (1: 500) including Goat Anti-Mouse IgG2a Alexa Fluor 647 (to detect dystrophin), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect SC-71), and Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5). Dystrophin, BA-D5, and SC-71 staining was pseudo colored white, red, and green, respectively. B: Serial cross-sections from the same bovine longissimus dorsi muscle biopsy sample shown in panel A were stained with a primary antibody against dystrophin [MANDYS1(3B7)s, MIgG2a, 1:10] to label the label the myofiber boundaries in combination with a cocktail of primary antibodies against MyHC type IIX (6H1s, MIgM, 1:10) and all MyHC isoforms except for type IIX (BF-35c, MIgG1, 1:100). Primary antibody staining was visualized using Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Mouse IgG2a Alexa Fluor 647 (to detect dystrophin), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35), and Goat Anti-Mouse IgM Alexa Fluor 555 (to detect 6H1). Dystrophin, BF-35, and 6H1 staining were pseudo colored white, red, and green, respectively. Scale bars are 200 µm.

Techniques: Staining

Journal: bioRxiv

Article Title: Rapid High-Throughput Analysis of Bovine Skeletal Muscle Fiber Morphology via Automated Fluorescent Microscopy and MuscleBos software

doi: 10.64898/2025.12.18.695234

Figure Lengend Snippet: Cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries, together with a cocktail of mouse monoclonal primary antibodies against MyHC type I (BA-D5, MIgG2b), MyHC type IIX (6H1, MIgM), and all MyHC isoforms except type IIX (BF-35, MIgG1). Primary antibody binding was visualized with Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 350 (to detect laminin), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), and Goat Anti-Mouse IgM Alexa Fluor 647 (to detect 6H1). Laminin, BA-D5, BF-35, and 6H1 staining were pseudo colored white, red, green, and blue, respectively. Scale bars are 100 µm.

Techniques: Staining, Binding Assay

Serial muscle cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries together with mouse monoclonal primary antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100), MyHC type IIX (6H1s, MIgM, 1:10), and all MyHC isoforms except type IIX (BF-35c, MIgG1, 1:100), applied either individually or in combination as a cocktail. Primary antibody staining was visualized with a cocktail of Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit 350 (to detect laminin), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), Goat Anti-Mouse IgM Alexa Fluor 647 (to detect 6H1), and Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35). Laminin, BA-D5, BF-35, and 6H1 were pseudo colored white, red, green, and blue, respectively. Scale bars are 100 µm.

Journal: bioRxiv

Article Title: Rapid High-Throughput Analysis of Bovine Skeletal Muscle Fiber Morphology via Automated Fluorescent Microscopy and MuscleBos software

doi: 10.64898/2025.12.18.695234

Figure Lengend Snippet: Serial muscle cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries together with mouse monoclonal primary antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100), MyHC type IIX (6H1s, MIgM, 1:10), and all MyHC isoforms except type IIX (BF-35c, MIgG1, 1:100), applied either individually or in combination as a cocktail. Primary antibody staining was visualized with a cocktail of Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit 350 (to detect laminin), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), Goat Anti-Mouse IgM Alexa Fluor 647 (to detect 6H1), and Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35). Laminin, BA-D5, BF-35, and 6H1 were pseudo colored white, red, green, and blue, respectively. Scale bars are 100 µm.

Techniques: Staining

A: Cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries in combination with a mouse monoclonal primary antibody against MyHC type I (BA-D5c, MIgG2b, 1:100). Primary antibody binding was visualized using Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 647 (to detect laminin) and Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5). Laminin and BA-D5 staining was pseudo colored white and red, respectively. Stitched panoramic images of entire muscle biopsy cross-sections were obtained using an automated fluorescent microscope (Echo Revolution) and analyzed using the MuscleJ 1.0.2 plugin for FIJI/ImageJ with minor custom modification. Segmented myofiber regions of interest (ROIs) were overlaid on the original images and corresponding cartography maps of type I fibers (red) versus non-type I (e.g., type II) fibers (gray) are shown. Scale bars are 400 µm on stitched mosaic images and 100 µm on representative fields of view. B: Comparison of total myofiber count data between manual image analysis and MuscleJ. C: Comparison of the percentage (%) composition of type I (BA-D5 pos ) fibers between manual image analysis and MuscleJ at different sensitivity thresholds for type I fiber identification. D: Comparison of the percentage (%) composition of type II myofibers (BA-D5 neg ) fibers between manual image analysis and MuscleJ at different sensitivity thresholds for type I fiber identification. B-D: Data is presented as the mean ± SEM of muscle biopsy samples from n=13 animals. ** and **** denote p<0.01 and p<0.0001 between the indicated groups, respectively.

Journal: bioRxiv

Article Title: Rapid High-Throughput Analysis of Bovine Skeletal Muscle Fiber Morphology via Automated Fluorescent Microscopy and MuscleBos software

doi: 10.64898/2025.12.18.695234

Figure Lengend Snippet: A: Cross-sections (10 µm) of bovine longissimus dorsi muscle biopsy samples were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries in combination with a mouse monoclonal primary antibody against MyHC type I (BA-D5c, MIgG2b, 1:100). Primary antibody binding was visualized using Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 647 (to detect laminin) and Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5). Laminin and BA-D5 staining was pseudo colored white and red, respectively. Stitched panoramic images of entire muscle biopsy cross-sections were obtained using an automated fluorescent microscope (Echo Revolution) and analyzed using the MuscleJ 1.0.2 plugin for FIJI/ImageJ with minor custom modification. Segmented myofiber regions of interest (ROIs) were overlaid on the original images and corresponding cartography maps of type I fibers (red) versus non-type I (e.g., type II) fibers (gray) are shown. Scale bars are 400 µm on stitched mosaic images and 100 µm on representative fields of view. B: Comparison of total myofiber count data between manual image analysis and MuscleJ. C: Comparison of the percentage (%) composition of type I (BA-D5 pos ) fibers between manual image analysis and MuscleJ at different sensitivity thresholds for type I fiber identification. D: Comparison of the percentage (%) composition of type II myofibers (BA-D5 neg ) fibers between manual image analysis and MuscleJ at different sensitivity thresholds for type I fiber identification. B-D: Data is presented as the mean ± SEM of muscle biopsy samples from n=13 animals. ** and **** denote p<0.01 and p<0.0001 between the indicated groups, respectively.

Techniques: Staining, Binding Assay, Microscopy, Modification, Comparison

Bovine longissimus dorsi muscle biopsy cross-sections were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries together with a cocktail of mouse monoclonal primary antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100), all but IIX (BF-35c, MIgG1, 1:100), and type IIX (6H1s, IgM, 1:10). Primary antibody binding was detected using Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 350 (to detect laminin), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35), and Goat Anti-Mouse IgM Alexa Fluor 647 (to detect 6H1). Laminin, BA-D5, SC-71, and 6H1 staining was pseudo colored white, red, green, and blue, respectively. Stitched panoramic images of the entire muscle biopsy cross-section were obtained using an automated fluorescent microscope (Echo Revolution) and analyzed using the MuscleJ plugin for FIJI/ImageJ with custom modifications. Original images and fiber type cartography maps of type I (red), type IIA (green), and type IIX (gray) are shown. Scale bars are 400 µm on stitched mosaic images and 100 µm on representative fields of view.

Journal: bioRxiv

Article Title: Rapid High-Throughput Analysis of Bovine Skeletal Muscle Fiber Morphology via Automated Fluorescent Microscopy and MuscleBos software

doi: 10.64898/2025.12.18.695234

Figure Lengend Snippet: Bovine longissimus dorsi muscle biopsy cross-sections were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries together with a cocktail of mouse monoclonal primary antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100), all but IIX (BF-35c, MIgG1, 1:100), and type IIX (6H1s, IgM, 1:10). Primary antibody binding was detected using Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 350 (to detect laminin), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35), and Goat Anti-Mouse IgM Alexa Fluor 647 (to detect 6H1). Laminin, BA-D5, SC-71, and 6H1 staining was pseudo colored white, red, green, and blue, respectively. Stitched panoramic images of the entire muscle biopsy cross-section were obtained using an automated fluorescent microscope (Echo Revolution) and analyzed using the MuscleJ plugin for FIJI/ImageJ with custom modifications. Original images and fiber type cartography maps of type I (red), type IIA (green), and type IIX (gray) are shown. Scale bars are 400 µm on stitched mosaic images and 100 µm on representative fields of view.

Techniques: Staining, Binding Assay, Microscopy

A: Bovine longissimus dorsi muscle cross-section (10 µm) were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries together with a cocktail of mouse monoclonal antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100), MyHC type IIX (6H1s, MIgM, 1:10), and all MyHC types except IIX (BF-35c, MIgG1, 1:100). Primary antibody staining was visualized with Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 350 (to detect laminin), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35), and Goat Anti-Mouse IgM Alexa Fluor 647 (to detect 6H1). Laminin, BA-D5, BF-35, and 6H1 were pseudo colored white, red, green, and blue, respectively. Stitched panoramic images of the entire muscle biopsy cross-section were obtained using an automated fluorescent microscope (Echo Revolution) and analyzed using the MuscleBos plugin for FIJI/ImageJ. An original image and corresponding manually or MuscleBos generated fiber type cartography maps are shown. Scale bars on representative fields of view are 100 µm. B–E: Comparison of manual and MuscleBos analysis of the same bovine muscle cross-sections for total myofiber count ( B ), the percentage of type I myofibers ( C ), the percentage of type IIA myofibers ( D ), and the percentage of type IIX myofibers ( E ). F–H: Comparison of manual and MuscleBos analysis of the same bovine muscle cross-sections for mean myofiber cross-sectional area (CSA) measurements for all fibers irrespective of type ( F ), type I fibers ( G ), type IIA fibers ( H ), and type IIX fibers ( I ). Dot plots represent data from each individual animal (biological replicates) for n=13 cows. NS denotes no significant difference between manual analysis and MuscleBos. * and ** denote p<0.05 and p<0.01 between the indicated groups, respectively.

Journal: bioRxiv

Article Title: Rapid High-Throughput Analysis of Bovine Skeletal Muscle Fiber Morphology via Automated Fluorescent Microscopy and MuscleBos software

doi: 10.64898/2025.12.18.695234

Figure Lengend Snippet: A: Bovine longissimus dorsi muscle cross-section (10 µm) were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries together with a cocktail of mouse monoclonal antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100), MyHC type IIX (6H1s, MIgM, 1:10), and all MyHC types except IIX (BF-35c, MIgG1, 1:100). Primary antibody staining was visualized with Alexa Fluor-conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 350 (to detect laminin), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35), and Goat Anti-Mouse IgM Alexa Fluor 647 (to detect 6H1). Laminin, BA-D5, BF-35, and 6H1 were pseudo colored white, red, green, and blue, respectively. Stitched panoramic images of the entire muscle biopsy cross-section were obtained using an automated fluorescent microscope (Echo Revolution) and analyzed using the MuscleBos plugin for FIJI/ImageJ. An original image and corresponding manually or MuscleBos generated fiber type cartography maps are shown. Scale bars on representative fields of view are 100 µm. B–E: Comparison of manual and MuscleBos analysis of the same bovine muscle cross-sections for total myofiber count ( B ), the percentage of type I myofibers ( C ), the percentage of type IIA myofibers ( D ), and the percentage of type IIX myofibers ( E ). F–H: Comparison of manual and MuscleBos analysis of the same bovine muscle cross-sections for mean myofiber cross-sectional area (CSA) measurements for all fibers irrespective of type ( F ), type I fibers ( G ), type IIA fibers ( H ), and type IIX fibers ( I ). Dot plots represent data from each individual animal (biological replicates) for n=13 cows. NS denotes no significant difference between manual analysis and MuscleBos. * and ** denote p<0.05 and p<0.01 between the indicated groups, respectively.

Techniques: Staining, Bioprocessing, Microscopy, Generated, Comparison

Journal: bioRxiv

Article Title: Rapid High-Throughput Analysis of Bovine Skeletal Muscle Fiber Morphology via Automated Fluorescent Microscopy and MuscleBos software

doi: 10.64898/2025.12.18.695234

Figure Lengend Snippet: (A): Muscle biopsy samples were obtained from the longissimus dorsi of high muscle (HM) or low muscle (LM) multiparous dairy cows at 21-days before expected calving (PRE) and at 21-days postpartum (POST). Biopsy cross-sections (10 µm) were stained with a primary antibody against laminin (ab7463, Rabbit, 1:100) to label the myofiber boundaries together with a cocktail of mouse monoclonal antibodies against MyHC type I (BA-D5c, MIgG2b, 1:100), MyHC type IIX (6H1s, MIgM, 1:10), and all MyHC types except IIX (BF-35c, MIgG1, 1:10). Primary antibody staining was visualized with Alexa Fluor conjugated secondary antibodies (1:500) including Goat Anti-Rabbit Alexa Fluor 350 (to detect laminin), Goat Anti-Mouse IgG2b Alexa Fluor 555 (to detect BA-D5), Goat Anti-Mouse IgG1 Alexa Fluor 488 (to detect BF-35), and Goat Anti-Mouse IgM Alexa Fluor 647 (to detect 6H1). Laminin, BA-D5, BF-35, and 6H1 were pseudo colored white, red, green, and blue, respectively. Scale bars are 100 µm. B-J: Stitched panoramic images of the entire muscle biopsy cross-section were obtained using an automated fluorescent microscope (Echo Revolution) and analyzed using the MuscleBos plugin for FIJI/ImageJ. B: The overall fiber type profile of pooled bovine longissimus dorsi muscle biopsy samples showing the percentage composition of type I, type IIA, and type IIX fibers as determined by MuscleBos. C: The mean cross-sectional area (CSA) type I, type IIA, and type IIX myofibers in pooled bovine longissimus dorsi muscle biopsy samples as determined by MuscleBos. D: Comparison of mean myofiber CSA irrespective of fiber type between bovine longissimus dorsi muscle biopsy samples obtained from HM or LM cows at PRE and POST parturition. E-G : Comparison of the mean CSA of type I (E) , type IIA (F) , and type IIX (G) myofibers in bovine longissimus dorsi muscle biopsy samples obtained from HM or LM cows at PRE and POST parturition H-J : Comparison of percentage fiber type composition of type I (H) , type IIA (I) , and type IIX (J) myofibers in bovine longissimus dorsi muscle biopsy samples obtained from HM or LM cows PRE and POST parturition. Values are mean ± SEM with dot plots representing data obtained from each individual animal (biological replicates). Symbols denote *p<0.05, **p<0.01, and ***p<0.001, and ****p<0.0001 between the indicated groups.

Techniques: Staining, Bioprocessing, Microscopy, Comparison

Ighmbp2 R604X/R604X mice showed changes in lung pathology and evidence of milk aspiration. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). Five mice for each genotype and three images per mouse were analyzed. (A) Thickening of the alveolar wall was scored 0–18 points. Each image/mouse was scored 0–6 with 0 = no thickening of alveolar wall (1 cell layer thick), 1–2 = mild thickening, 3–4 = moderate thickening of alveolar wall, 5 = extensive thickening of alveolar wall, 6 = complete thickening. Representative image of thickening of alveolar wall. Mean wild type = 3.2, Ighmbp2 +/R604X = 3.7, Ighmbp2 R604X/R604X = 8.8. +/+ and R604X/R604X, P = 0.0007; +/R604X and R604X/R604X, P = 0.0010, DF = 13 (F(DFn,DFd) F(2,13) = 15.7, P = 0.0003). (B) The presence of hyaline membrane (acellular deposits in the alveolar region) was scored 0–6 points. Each image/mouse was scored 0–2 with 0 = no acellular debris, 1 = partial build up of acellular debris, 2 = significant build up of acellular debris. Representative image of hyaline membrane. Mean wild type = 0.0, Ighmbp2 +/R604X = 0.5, Ighmbp2 R604X/R604X = 1.8. +/+ and R604X/R604X, P = 0.0091; +/R604X and R604X/R604X, P = 0.0460, DF = 13 (F(DFn,DFd) F(2,13) = 6.8, P = 0.0095). (C) Observed enhanced injury was scored 0–18 points. Each image/mouse was scored 0–6 with 0 = no damage, 1 = minimum damage, 2 = mild damage, 3 = moderate damage, 4 = pronounced damage, 5 = extensive damage, 6 = completely damaged. Representative image of enhanced injury in lung. Mean wild type = 2.0, Ighmbp2 +/R604X = 3.3, Ighmbp2 R604X/R604X = 11.2. +/+ and R604X/R604X, P < 0.0001; +/R604X and R604X/R604X, P < 0.0001, DF = 13 (F(DFn,DFd) F(2,13) = 38.8, P < 0.0001). (D) Atelectasis (complete or partial collapse of distal air spaces) was scored 0–12. Each image/mouse was scored 0–4 with 0 = no collapse, 1 = slight collapse, 2 = 50 % collapse, 3 = nearly full 75 % collapse, 4 = full collapse of distal air spaces. Representative image of atelectasis within the lung. Mean wild type = 1.2, Ighmbp2 +/R604X = 1.7, Ighmbp2 R604X/R604X = 5.2. +/+ and R604X/R604X, P = 0.0004, +/R604X and R604X/R604X, P = 0.0009, DF = 13 (F(DFn,DFd) F (2,13) = 16.8, P = 0.0003). (E) Representative images of lungs from wild type, Ighmbp2 +/R604X , and Ighmbp2 R604X/R604X mice lungs immunostained with antibodies against milk proteins (red-see white arrows). The presence of milk within an alveoli was scored based on the presence of anti-milk antibody staining. Five mice were scored per genotype with wild type (708 alveoli scored, 118+ alveoli/mouse), Ighmbp2 +/R604X (683 alveoli scored, 102+ alveoli/mouse), and Ighmbp2 R604X/R604X (593 alveoli scored, 91 + alveoli/mouse). Wild type (mean = 1.7 %), Ighmbp2 +/R604X (mean = 2.9 %), and Ighmbp2 R604X/R604X (12.1 %) (* P = 0.0134, ** P = 0.0064) (F(DFn,DFd) F(2,12) = 8.8, P = 0.0045). Statistical analyses: one-way ANOVA with Tukey’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Journal: Neurobiology of disease

Article Title: The Ighmbp2 -R604X mouse presents with the most severe SMARD1 clinical symptoms resulting in failure to thrive, respiratory and feeding deficits, aspiration and severe axon and muscle pathology

doi: 10.1016/j.nbd.2025.107199

Figure Lengend Snippet: Ighmbp2 R604X/R604X mice showed changes in lung pathology and evidence of milk aspiration. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). Five mice for each genotype and three images per mouse were analyzed. (A) Thickening of the alveolar wall was scored 0–18 points. Each image/mouse was scored 0–6 with 0 = no thickening of alveolar wall (1 cell layer thick), 1–2 = mild thickening, 3–4 = moderate thickening of alveolar wall, 5 = extensive thickening of alveolar wall, 6 = complete thickening. Representative image of thickening of alveolar wall. Mean wild type = 3.2, Ighmbp2 +/R604X = 3.7, Ighmbp2 R604X/R604X = 8.8. +/+ and R604X/R604X, P = 0.0007; +/R604X and R604X/R604X, P = 0.0010, DF = 13 (F(DFn,DFd) F(2,13) = 15.7, P = 0.0003). (B) The presence of hyaline membrane (acellular deposits in the alveolar region) was scored 0–6 points. Each image/mouse was scored 0–2 with 0 = no acellular debris, 1 = partial build up of acellular debris, 2 = significant build up of acellular debris. Representative image of hyaline membrane. Mean wild type = 0.0, Ighmbp2 +/R604X = 0.5, Ighmbp2 R604X/R604X = 1.8. +/+ and R604X/R604X, P = 0.0091; +/R604X and R604X/R604X, P = 0.0460, DF = 13 (F(DFn,DFd) F(2,13) = 6.8, P = 0.0095). (C) Observed enhanced injury was scored 0–18 points. Each image/mouse was scored 0–6 with 0 = no damage, 1 = minimum damage, 2 = mild damage, 3 = moderate damage, 4 = pronounced damage, 5 = extensive damage, 6 = completely damaged. Representative image of enhanced injury in lung. Mean wild type = 2.0, Ighmbp2 +/R604X = 3.3, Ighmbp2 R604X/R604X = 11.2. +/+ and R604X/R604X, P < 0.0001; +/R604X and R604X/R604X, P < 0.0001, DF = 13 (F(DFn,DFd) F(2,13) = 38.8, P < 0.0001). (D) Atelectasis (complete or partial collapse of distal air spaces) was scored 0–12. Each image/mouse was scored 0–4 with 0 = no collapse, 1 = slight collapse, 2 = 50 % collapse, 3 = nearly full 75 % collapse, 4 = full collapse of distal air spaces. Representative image of atelectasis within the lung. Mean wild type = 1.2, Ighmbp2 +/R604X = 1.7, Ighmbp2 R604X/R604X = 5.2. +/+ and R604X/R604X, P = 0.0004, +/R604X and R604X/R604X, P = 0.0009, DF = 13 (F(DFn,DFd) F (2,13) = 16.8, P = 0.0003). (E) Representative images of lungs from wild type, Ighmbp2 +/R604X , and Ighmbp2 R604X/R604X mice lungs immunostained with antibodies against milk proteins (red-see white arrows). The presence of milk within an alveoli was scored based on the presence of anti-milk antibody staining. Five mice were scored per genotype with wild type (708 alveoli scored, 118+ alveoli/mouse), Ighmbp2 +/R604X (683 alveoli scored, 102+ alveoli/mouse), and Ighmbp2 R604X/R604X (593 alveoli scored, 91 + alveoli/mouse). Wild type (mean = 1.7 %), Ighmbp2 +/R604X (mean = 2.9 %), and Ighmbp2 R604X/R604X (12.1 %) (* P = 0.0134, ** P = 0.0064) (F(DFn,DFd) F(2,12) = 8.8, P = 0.0045). Statistical analyses: one-way ANOVA with Tukey’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Article Snippet: Tissues were cryosectioned in 10 μm sections were co-stained with anti-Laminin primary antibody (1:200; catalog: L9393; Millipore Sigma), myosin heavy chain (MyHC) type 1 (1:10, catalog: BA-D5-s, Iowa Developmental Studies Hybridoma Bank), MyHC type 2 A (1:50, catalog: SC-71-s, Iowa Developmental Studies Hybridoma Bank), MyHC type 2B (1:5, catalog: BF-F3-s, Iowa Developmental Studies Hybridoma Bank).

Techniques: Membrane, Staining

Diaphragm muscle fiber type composition was not altered but muscle fibers were smaller in Ighmbp2 R604X/R604X mice. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). (A) Diaphragm muscle fiber area (+/+ = 365 μm 2 , +/R604X = 358 μm 2 , R604X/R604X = 262 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 8044) (F(DFn,DFd) F(2,8044) = 335.8, P < 0.0001). (B) Diaphragm embryonic muscle fiber area (+/+ = 449 μm 2 , +/R604X = 363 μm 2 , R604X/R604X = 228 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/+ and +/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 4771) (F(DFn,DFd) F(2,4771) = 566.3, P < 0.0001). (C) Diaphragm Type 1 muscle fiber area (+/+ = 396 μm 2 , +/R604X = 391 μm 2 , R604X/R604X = 242 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 417) (F(DFn,DFd) F(2,417) = 93.4, P < 0.0001). (D) Diaphragm Type 2 A muscle fiber area (+/+ = 374 μm 2 , +/R604X = 347 μm 2 , R604X/R604X = 231 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X; P = 0.0393, +/+ and +/R604X, DF = 1148) (F(DFn,DFd) F(2,1148) = 112.4, P < 0.0001). (E) Diaphragm Type 2B muscle fiber area (+/+ = 437 μm 2 , +/R604X = 465 μm 2 , R604X/R604X = 329 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 1232) (F (DFn,DFd) F(2,1232) = 49.7, P < 0.0001) (F) Diaphragm non-labelled (representing embryonic and Type X) muscle fiber area (+/+ = 431 μm 2 , +/R604X = 445 μm 2 , R604X/R604X = 290 μm 2 , P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 2229) (F(DFn,DFd) F(2,2229) = 201.9, P < 0.0001) (G) Diaphragm Type 1 + 2 A hybrid muscle fiber area (+/+ = 352 μm 2 , +/R604X = 309 μm 2 , R604X/R604X = 201 μm 2 , P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 213) (F(DFn,DFd) F(2,213) = 33.7, P < 0.0001). (H) Diaphragm Type 1 + 2B hybrid muscle fiber area (+/+ = 317 μm 2 , +/R604X = 337 μm 2 , R604X/R604X = 280 μm 2 ), P = 0.0381, (F(DFn,DFd) F(2,207) = 3.7, P = 0.0269). (I) Diaphragm Type 1 + 2 A + 2B muscle fiber area (+/+ = 238 μm 2 , +/R604X = 242 μm 2 , R604X/R604X = 215 μm 2 ; P = 0.0060, +/+ and R604X/R604X; P = 0.0003, +/R604X and R604X/R604X, DF = 1402) (F(DFn,DFd) F(2,1402) = 9, P = 0.0001). (J) Frequency distribution of diaphragm muscle fiber area in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice. (K) Percentage of all cells expressing a given muscle fiber type in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice, P = 0.0491. Five mice from each genotype (15 mice total) were analyzed with 2434–3177 total muscle fibers analyzed per genotype. Statistical analyses: one-way ANOVA with Dunnett’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Journal: Neurobiology of disease

doi: 10.1016/j.nbd.2025.107199

Figure Lengend Snippet: Diaphragm muscle fiber type composition was not altered but muscle fibers were smaller in Ighmbp2 R604X/R604X mice. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). (A) Diaphragm muscle fiber area (+/+ = 365 μm 2 , +/R604X = 358 μm 2 , R604X/R604X = 262 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 8044) (F(DFn,DFd) F(2,8044) = 335.8, P < 0.0001). (B) Diaphragm embryonic muscle fiber area (+/+ = 449 μm 2 , +/R604X = 363 μm 2 , R604X/R604X = 228 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/+ and +/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 4771) (F(DFn,DFd) F(2,4771) = 566.3, P < 0.0001). (C) Diaphragm Type 1 muscle fiber area (+/+ = 396 μm 2 , +/R604X = 391 μm 2 , R604X/R604X = 242 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 417) (F(DFn,DFd) F(2,417) = 93.4, P < 0.0001). (D) Diaphragm Type 2 A muscle fiber area (+/+ = 374 μm 2 , +/R604X = 347 μm 2 , R604X/R604X = 231 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X; P = 0.0393, +/+ and +/R604X, DF = 1148) (F(DFn,DFd) F(2,1148) = 112.4, P < 0.0001). (E) Diaphragm Type 2B muscle fiber area (+/+ = 437 μm 2 , +/R604X = 465 μm 2 , R604X/R604X = 329 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 1232) (F (DFn,DFd) F(2,1232) = 49.7, P < 0.0001) (F) Diaphragm non-labelled (representing embryonic and Type X) muscle fiber area (+/+ = 431 μm 2 , +/R604X = 445 μm 2 , R604X/R604X = 290 μm 2 , P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 2229) (F(DFn,DFd) F(2,2229) = 201.9, P < 0.0001) (G) Diaphragm Type 1 + 2 A hybrid muscle fiber area (+/+ = 352 μm 2 , +/R604X = 309 μm 2 , R604X/R604X = 201 μm 2 , P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 213) (F(DFn,DFd) F(2,213) = 33.7, P < 0.0001). (H) Diaphragm Type 1 + 2B hybrid muscle fiber area (+/+ = 317 μm 2 , +/R604X = 337 μm 2 , R604X/R604X = 280 μm 2 ), P = 0.0381, (F(DFn,DFd) F(2,207) = 3.7, P = 0.0269). (I) Diaphragm Type 1 + 2 A + 2B muscle fiber area (+/+ = 238 μm 2 , +/R604X = 242 μm 2 , R604X/R604X = 215 μm 2 ; P = 0.0060, +/+ and R604X/R604X; P = 0.0003, +/R604X and R604X/R604X, DF = 1402) (F(DFn,DFd) F(2,1402) = 9, P = 0.0001). (J) Frequency distribution of diaphragm muscle fiber area in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice. (K) Percentage of all cells expressing a given muscle fiber type in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice, P = 0.0491. Five mice from each genotype (15 mice total) were analyzed with 2434–3177 total muscle fibers analyzed per genotype. Statistical analyses: one-way ANOVA with Dunnett’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Techniques: Expressing

Tricep muscle fiber size and composition were altered in Ighmbp2 R604X/R604X mice. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). (A) Tricep muscle fiber area (+/+ = 465 μm 2 , +/R604X = 373 μm 2 , R604X/R604X = 267 μm 2 ; P < 0.0001, DF = 17,254) (F(DFn,DFd) F(2,17,254) = 870, P < 0.0001). (B) Tricep embryonic muscle fiber area (+/+ = 301 μm 2 , +/R604X = 235 μm 2 , R604X/R604X = 184 μm 2 ; P < 0.0001, DF = 5073) (F(DFn,DFd) F (2,5073) = 108.1, P < 0.0001). (C) Tricep Type 1 muscle fiber area (+/+ = 324 μm 2 , +/R604X = 250 μm 2 , R604X/R604X = 280 μm 2 ; P = 0.0018, +/+ and R604X/R604X; P = 0.0023, +/+ and +/R604X, DF = 616) (F(DFn,DFd) F(2,616) = 8.2, P = 0.0003). (D) Tricep Type 2 A muscle fiber area (+/+ = 177 μm 2 , +/R604X = 168 μm 2 , R604X/R604X = 164 μm 2 ) (F(DFn,DFd) F(2,648) = 2.3, P = 0.1024). (E) Tricep Type 2B muscle fiber area (+/+ = 614 μm 2 , +/R604X = 436 μm 2 , R604X/R604X = 588 μm 2 ; P < 0.0001, +/+ and +/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 6068) (F(DFn,DFd) F(2,6068) = 240.3, P < 0.0001). (F) Tricep non-labelled (representing embryonic and Type X) muscle fiber area (+/+ = 359 μm 2 , +/R604X = 296 μm 2 , R604X/R604X = 268 μm 2 ; P < 0.0001, DF = 9146) (F (DFn,DFd) F(2,9196) = 167.2, P < 0.0001). (G) Percentage of all cells expressing a given muscle fiber type in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice (embryonic +/+ = 6, +/R604X = 12, R604X/R604X = 35; P < 0.0001, +/+ and R604X/R604X; P = 0.0004, +/R604X and R604X/R604X; DF = 11) (Type 2B +/+ = 49, +/R604X = 62, R604X/R604X = 2; P < 0.0001, P = 0.0117, +/+ and +/R604X) (non-labelled +/+ = 37, +/R604X = 28, R604X/R604X = 86; P < 0.0001, DF = 88). Four to five mice from each genotype were analyzed (14 mice total). Statistical analyses: one-way ANOVA with Dunnett’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Journal: Neurobiology of disease

doi: 10.1016/j.nbd.2025.107199

Figure Lengend Snippet: Tricep muscle fiber size and composition were altered in Ighmbp2 R604X/R604X mice. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). (A) Tricep muscle fiber area (+/+ = 465 μm 2 , +/R604X = 373 μm 2 , R604X/R604X = 267 μm 2 ; P < 0.0001, DF = 17,254) (F(DFn,DFd) F(2,17,254) = 870, P < 0.0001). (B) Tricep embryonic muscle fiber area (+/+ = 301 μm 2 , +/R604X = 235 μm 2 , R604X/R604X = 184 μm 2 ; P < 0.0001, DF = 5073) (F(DFn,DFd) F (2,5073) = 108.1, P < 0.0001). (C) Tricep Type 1 muscle fiber area (+/+ = 324 μm 2 , +/R604X = 250 μm 2 , R604X/R604X = 280 μm 2 ; P = 0.0018, +/+ and R604X/R604X; P = 0.0023, +/+ and +/R604X, DF = 616) (F(DFn,DFd) F(2,616) = 8.2, P = 0.0003). (D) Tricep Type 2 A muscle fiber area (+/+ = 177 μm 2 , +/R604X = 168 μm 2 , R604X/R604X = 164 μm 2 ) (F(DFn,DFd) F(2,648) = 2.3, P = 0.1024). (E) Tricep Type 2B muscle fiber area (+/+ = 614 μm 2 , +/R604X = 436 μm 2 , R604X/R604X = 588 μm 2 ; P < 0.0001, +/+ and +/R604X; P < 0.0001, +/R604X and R604X/R604X, DF = 6068) (F(DFn,DFd) F(2,6068) = 240.3, P < 0.0001). (F) Tricep non-labelled (representing embryonic and Type X) muscle fiber area (+/+ = 359 μm 2 , +/R604X = 296 μm 2 , R604X/R604X = 268 μm 2 ; P < 0.0001, DF = 9146) (F (DFn,DFd) F(2,9196) = 167.2, P < 0.0001). (G) Percentage of all cells expressing a given muscle fiber type in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice (embryonic +/+ = 6, +/R604X = 12, R604X/R604X = 35; P < 0.0001, +/+ and R604X/R604X; P = 0.0004, +/R604X and R604X/R604X; DF = 11) (Type 2B +/+ = 49, +/R604X = 62, R604X/R604X = 2; P < 0.0001, P = 0.0117, +/+ and +/R604X) (non-labelled +/+ = 37, +/R604X = 28, R604X/R604X = 86; P < 0.0001, DF = 88). Four to five mice from each genotype were analyzed (14 mice total). Statistical analyses: one-way ANOVA with Dunnett’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Techniques: Expressing

TA muscle fiber size and types were altered in Ighmbp2 R604X/R604X mice. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). (A) TA total muscle fiber area (+/+ = 393 μm 2 , +/R604X = 404 μm 2 , R604X/R604X = 332 μm 2 , P < 0.0001, DF = 20,509) (F(DFn,DFd) F(2,2050) = 165.9, P < 0.0001). (B) TA embryonic muscle fiber area (+/+ = 253 μm 2 , +/R604X = 286 μm 2 , R604X/R604X = 306 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/+ and +/R604X; P = 0.0001, +/R604X and R604X/R604X, DF = 10,998) (F(DFn,DFd) F(2,10,998) = 54.1, P < 0.0001). (C) TA Type 1 muscle fiber area (+/+ = 373 μm 2 , +/R604X = 400 μm 2 , R604X/R604X = 311 μm 2 ; P < 0.0001, P < 0.0001, DF = 1596) (F(DFn,DFd) F(2,1596) = 37.8, P < 0.0001). (D) TA Type 2 A muscle fiber area (+/+ = 296 μm 2 , +/R604X = 298 μm 2 , R604X/R604X = 183 μm 2 ; P < 0.0001, DF = 1333) (F(DFn,DFd) F(2,1333) = 212, P < 0.0001). (E) TA Type 2B muscle fiber area (+/+ = 570 μm 2 , +/R604X = 753 μm 2 , R604X/R604X = 534 μm 2 ; P < 0.0001, +/+ and +/R604X; P < 0.0001, +/R604X and R604X/R604X, P = 0.0252, +/+ and R604X/R604X, DF = 3307) (F(DFn,DFd) F(2,3307) = 159.1, P < 0.0001). (F) TA non-labelled (representing embryonic and Type X) muscle fiber area (+/+ = 402 μm 2 , +/R604X = 469 μm 2 , R604X/R604X = 341 μm 2 ; P < 0.0001, +/+ and +/R604X; P < 0.0001, +/R604X and R604X/R604X; P < 0.0001, +/+ and R604X/R604X, DF = 10,295) (F(DFn,DFd) F(2,10,295) = 293.1, P < 0.0001). (G) Percentage of all cells expressing a given muscle fiber type in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice (embryonic +/+ = 32, +/R604X = 35, R604X/R604X = 66; P = 0.0002, +/+ and R604X/R604X; P = 0.0005, +/R604X and R604X/R604X, DF = 12) (Type 2B +/+ = 45, +/R604X = 27, R604X/R604X = 8, P < 0.0001.) (non-labelled +/+ = 36, +/R604X = 55, R604X/R604X = 69, P < 0.0001). Five mice from each genotype were analyzed (15 mice total). Statistical analyses: one-way ANOVA with Dunnett’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Journal: Neurobiology of disease

doi: 10.1016/j.nbd.2025.107199

Figure Lengend Snippet: TA muscle fiber size and types were altered in Ighmbp2 R604X/R604X mice. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). (A) TA total muscle fiber area (+/+ = 393 μm 2 , +/R604X = 404 μm 2 , R604X/R604X = 332 μm 2 , P < 0.0001, DF = 20,509) (F(DFn,DFd) F(2,2050) = 165.9, P < 0.0001). (B) TA embryonic muscle fiber area (+/+ = 253 μm 2 , +/R604X = 286 μm 2 , R604X/R604X = 306 μm 2 ; P < 0.0001, +/+ and R604X/R604X; P < 0.0001, +/+ and +/R604X; P = 0.0001, +/R604X and R604X/R604X, DF = 10,998) (F(DFn,DFd) F(2,10,998) = 54.1, P < 0.0001). (C) TA Type 1 muscle fiber area (+/+ = 373 μm 2 , +/R604X = 400 μm 2 , R604X/R604X = 311 μm 2 ; P < 0.0001, P < 0.0001, DF = 1596) (F(DFn,DFd) F(2,1596) = 37.8, P < 0.0001). (D) TA Type 2 A muscle fiber area (+/+ = 296 μm 2 , +/R604X = 298 μm 2 , R604X/R604X = 183 μm 2 ; P < 0.0001, DF = 1333) (F(DFn,DFd) F(2,1333) = 212, P < 0.0001). (E) TA Type 2B muscle fiber area (+/+ = 570 μm 2 , +/R604X = 753 μm 2 , R604X/R604X = 534 μm 2 ; P < 0.0001, +/+ and +/R604X; P < 0.0001, +/R604X and R604X/R604X, P = 0.0252, +/+ and R604X/R604X, DF = 3307) (F(DFn,DFd) F(2,3307) = 159.1, P < 0.0001). (F) TA non-labelled (representing embryonic and Type X) muscle fiber area (+/+ = 402 μm 2 , +/R604X = 469 μm 2 , R604X/R604X = 341 μm 2 ; P < 0.0001, +/+ and +/R604X; P < 0.0001, +/R604X and R604X/R604X; P < 0.0001, +/+ and R604X/R604X, DF = 10,295) (F(DFn,DFd) F(2,10,295) = 293.1, P < 0.0001). (G) Percentage of all cells expressing a given muscle fiber type in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice (embryonic +/+ = 32, +/R604X = 35, R604X/R604X = 66; P = 0.0002, +/+ and R604X/R604X; P = 0.0005, +/R604X and R604X/R604X, DF = 12) (Type 2B +/+ = 45, +/R604X = 27, R604X/R604X = 8, P < 0.0001.) (non-labelled +/+ = 36, +/R604X = 55, R604X/R604X = 69, P < 0.0001). Five mice from each genotype were analyzed (15 mice total). Statistical analyses: one-way ANOVA with Dunnett’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Techniques: Expressing

Ighmbp2 R604X/R604X mice showed reduced gastrocnemius muscle fiber size in all fibers but type I. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). (A) Gastrocnemius muscle fiber area (+/+ = 388 μm 2 , +/R604X = 273 μm 2 , R604X/R604X = 274 μm 2 ; P < 0.0001, DF14667) (F(DFn, DFd) F(2,14,667) = 577.2, P < 0.0001). (B) Gastrocnemius embryonic muscle fiber area (+/+ = 324 μm 2 , +/R604X = 241 μm 2 , R604X/R604X = 236 μm 2 ; P < 0.0001, DF = 8161) (F(DFn,DFd) F(2,8161) = 374.6, P < 0.0001). (C) Gastrocnemius Type 1 muscle fiber area (+/+ = 323 μm 2 , +/R604X = 302 μm 2 , R604X/R604X = 305 μm 2 ) (F(DFn,DFd) F(2,640) = 0.9, P = 0.3929). (D) Gastrocnemius Type 2 A muscle fiber area (+/+ = 342 μm 2 , +/R604X = 211 μm 2 , R604X/R604X = 227 μm 2 ; P = 0.0001, DF = 1767) (F(DFn,DFd) F(2,1767) = 121.4, P < 0.0001). (E) Gastrocnemius Type 2B muscle fiber area (+/+ = 501 μm 2 , +/R604X = 348 μm 2 , R604X/R604X = 299 μm 2 ; P < 0.0001, DF = 3602) (F(DFn,DFd) F(2,3602) = 298, P < 0.0001). (F) Gastrocnemius non-labelled (representing embryonic and Type X) muscle fiber area (+/+ = 363 μm 2 , +/R604X = 277 μm 2 , R604X/R604X = 292 μm 2 ; P < 0.0001, P = 0.0155, +/R604X and R604X/R604X; DF = 5201) (F (DFn,DFd) F(2,5201) = 84.2, P < 0.0001). (G) Percentage of all cells expressing a given muscle fiber type in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice (embryonic +/+ = 63, +/R604X = 47, R604X/R604X = 59) (non-labelled +/+ = 24, +/R604X = 30, R604X/R604X = 49; P = 0.0004 +/+ and R604X/R604X; P = 0.0133 +/R604X and R604X/R604X). Five mice from each genotype were analyzed (15 mice total). Statistical analyses: one-way ANOVA with Dunnett’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Journal: Neurobiology of disease

doi: 10.1016/j.nbd.2025.107199

Figure Lengend Snippet: Ighmbp2 R604X/R604X mice showed reduced gastrocnemius muscle fiber size in all fibers but type I. Wild type (black), Ighmbp2 +/R604X (orange), and Ighmbp2 R604X/R604X (blue). (A) Gastrocnemius muscle fiber area (+/+ = 388 μm 2 , +/R604X = 273 μm 2 , R604X/R604X = 274 μm 2 ; P < 0.0001, DF14667) (F(DFn, DFd) F(2,14,667) = 577.2, P < 0.0001). (B) Gastrocnemius embryonic muscle fiber area (+/+ = 324 μm 2 , +/R604X = 241 μm 2 , R604X/R604X = 236 μm 2 ; P < 0.0001, DF = 8161) (F(DFn,DFd) F(2,8161) = 374.6, P < 0.0001). (C) Gastrocnemius Type 1 muscle fiber area (+/+ = 323 μm 2 , +/R604X = 302 μm 2 , R604X/R604X = 305 μm 2 ) (F(DFn,DFd) F(2,640) = 0.9, P = 0.3929). (D) Gastrocnemius Type 2 A muscle fiber area (+/+ = 342 μm 2 , +/R604X = 211 μm 2 , R604X/R604X = 227 μm 2 ; P = 0.0001, DF = 1767) (F(DFn,DFd) F(2,1767) = 121.4, P < 0.0001). (E) Gastrocnemius Type 2B muscle fiber area (+/+ = 501 μm 2 , +/R604X = 348 μm 2 , R604X/R604X = 299 μm 2 ; P < 0.0001, DF = 3602) (F(DFn,DFd) F(2,3602) = 298, P < 0.0001). (F) Gastrocnemius non-labelled (representing embryonic and Type X) muscle fiber area (+/+ = 363 μm 2 , +/R604X = 277 μm 2 , R604X/R604X = 292 μm 2 ; P < 0.0001, P = 0.0155, +/R604X and R604X/R604X; DF = 5201) (F (DFn,DFd) F(2,5201) = 84.2, P < 0.0001). (G) Percentage of all cells expressing a given muscle fiber type in wild type, Ighmbp2 +/R604X and Ighmbp2 R604X/R604X mice (embryonic +/+ = 63, +/R604X = 47, R604X/R604X = 59) (non-labelled +/+ = 24, +/R604X = 30, R604X/R604X = 49; P = 0.0004 +/+ and R604X/R604X; P = 0.0133 +/R604X and R604X/R604X). Five mice from each genotype were analyzed (15 mice total). Statistical analyses: one-way ANOVA with Dunnett’s multiple comparisons. Values are expressed as mean. DF = degrees of freedom. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

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