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Fig. 4 | Acta Neuropathologica Communications

Fig. 4

From: Filamentous tangles with nemaline rods in MYH2 myopathy: a novel phenotype

Fig. 4

Immunofluorescence with confocal imaging identifies MyHC-IIX, MyHC-IIA,and IIA/IIX hybrid fibers. a Immunostaining for MyHC-IIX (orange) using the 6H1 antibody (mouse anti-human IgM, 1:1 dilution, Developmental Studies Hybridoma Bank (DSHB), and b for MyHC-IIA (green, intensely staining) and IIX proteins (weakly staining) (A4.74 antibody, 1:200 dilution) [16] are merged in c with DAPI nuclear staining. The series identifies a IIA fiber (arrows) and a hybrid IIA/IIX fiber (asterisk) that demonstrate filamentous changes and centralized myofiber disorganization. The IIX fibers display relatively normal size and architecture. d Confocal Z-stack imaging provides further detail of the 3-dimensional structure of the filamentous changes in the IIA fiber. Primary antibodies were visualized using Alexa Fluor® 488 conjugated goat anti-mouse IgG1 (A4.74) and Alexa Fluor® 555 conjugated goat anti-mouse IgM secondary antibodies at 1:200 dilutions (Invitrogen / ThermoFisher Scientific, Waltham, MA USA)

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