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Loss of C9orf72 induces muscle atrophy and alterations in the muscle fiber composition. Representative optical images of the GCM muscle in C9 +/+ ( A ) and C9 −/− ( B ) mice and myofibers mean cross-sectional area (CSA) distribution ( C ) in both experimental groups. Representative images of coronal sections of the GCM muscle in C9+/+ ( D ) and C9 −/− ( E ) labeled with laminin (white), myosin heavy chain <t>(MyHC)</t> type I (red), IIA (green), IIX (black), and IIB (blue). Analysis of the fiber type composition is shown in ( F ). Type IIX fibers correspond to unlabeled fibers. Representative electron micrographs of C9 +/+ ( G ) and C9 −/− ( H ) GCM muscles, respectively. * = slow (S) motor units. Data are expressed as mean± SEM of six mice per group and examined by two-way ANOVA followed by Fisher’s LSD test. * p < 0.05, ** p < 0.01, *** p < 0.001.
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Loss of C9orf72 induces muscle atrophy and alterations in the muscle fiber composition. Representative optical images of the GCM muscle in C9 +/+ ( A ) and C9 −/− ( B ) mice and myofibers mean cross-sectional area (CSA) distribution ( C ) in both experimental groups. Representative images of coronal sections of the GCM muscle in C9+/+ ( D ) and C9 −/− ( E ) labeled with laminin (white), myosin heavy chain <t>(MyHC)</t> type I (red), IIA (green), IIX (black), and IIB (blue). Analysis of the fiber type composition is shown in ( F ). Type IIX fibers correspond to unlabeled fibers. Representative electron micrographs of C9 +/+ ( G ) and C9 −/− ( H ) GCM muscles, respectively. * = slow (S) motor units. Data are expressed as mean± SEM of six mice per group and examined by two-way ANOVA followed by Fisher’s LSD test. * p < 0.05, ** p < 0.01, *** p < 0.001.
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Loss of C9orf72 induces muscle atrophy and alterations in the muscle fiber composition. Representative optical images of the GCM muscle in C9 +/+ ( A ) and C9 −/− ( B ) mice and myofibers mean cross-sectional area (CSA) distribution ( C ) in both experimental groups. Representative images of coronal sections of the GCM muscle in C9+/+ ( D ) and C9 −/− ( E ) labeled with laminin (white), myosin heavy chain (MyHC) type I (red), IIA (green), IIX (black), and IIB (blue). Analysis of the fiber type composition is shown in ( F ). Type IIX fibers correspond to unlabeled fibers. Representative electron micrographs of C9 +/+ ( G ) and C9 −/− ( H ) GCM muscles, respectively. * = slow (S) motor units. Data are expressed as mean± SEM of six mice per group and examined by two-way ANOVA followed by Fisher’s LSD test. * p < 0.05, ** p < 0.01, *** p < 0.001.

Journal: Cells

Article Title: C9ORF72 Is Pivotal to Maintain a Proper Protein Homeostasis in Mouse Skeletal Muscle

doi: 10.3390/cells14221765

Figure Lengend Snippet: Loss of C9orf72 induces muscle atrophy and alterations in the muscle fiber composition. Representative optical images of the GCM muscle in C9 +/+ ( A ) and C9 −/− ( B ) mice and myofibers mean cross-sectional area (CSA) distribution ( C ) in both experimental groups. Representative images of coronal sections of the GCM muscle in C9+/+ ( D ) and C9 −/− ( E ) labeled with laminin (white), myosin heavy chain (MyHC) type I (red), IIA (green), IIX (black), and IIB (blue). Analysis of the fiber type composition is shown in ( F ). Type IIX fibers correspond to unlabeled fibers. Representative electron micrographs of C9 +/+ ( G ) and C9 −/− ( H ) GCM muscles, respectively. * = slow (S) motor units. Data are expressed as mean± SEM of six mice per group and examined by two-way ANOVA followed by Fisher’s LSD test. * p < 0.05, ** p < 0.01, *** p < 0.001.

Article Snippet: To determine the fiber type, the sections were incubated with MyHC type I (BA-D5, 1:10; DSHB, Iowa City, IA, USA), MyHC type IIa (SC-71, 1:17; DSHB, Iowa City, IA, USA), MyHC type IIb (BF-F3, 1:9; DSHB, Iowa City, IA, USA), Rabbit anti-Laminin (1:100, L9393; Sigma-Aldrich, St. Louis, MO, USA) primary antibodies and respective secondary antibodies, anti-MIgG2b Alexa-flour 564 (A21144, 1:500) (Invitrogen, Waltham, MA, USA), anti-MIgG1 Alexa-flour 488 (A21121, 1:500) (Invitrogen, Waltham, MA, USA), anti-MIgM Alexa-flour 647 (A21046, 1:500; Invitrogen, Waltham, MA, USA), and anti-Rabbit Alexa Fluor 405 (ab175649, 1:500; Abcam, Cambridge, UK).

Techniques: Labeling, Muscles