General features Adult MTM1 Titin Juvenile DNM2 mutations Myopathic Muscle fibers Necklace fibers Abnormal fiber types Punctate, Small fibers Childhood Infant MTM1 |
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![]() From: T Mozaffar
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![]() H&E stain |
![]() H&E stain |
![]() H&E stain |
![]() H&E stain |
![]() Toluidine blue stain |
Central nuclei & abnormal internal architecture![]() Toluidine blue stain |
![]() Toluidine blue stain |
![]() Toluidine blue stain |
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![]() Electron microscopy from T Mozaffar |
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![]() ATPase stain, pH 9.4 |
![]() ATPase stain, pH 9.4 |
![]() NADH stain |
![]() NADH stain |
![]() ![]() ![]() NADH stain |
![]() H&E stain |
![]() H&E stain |
![]() NADH stain |
![]() ATPase stain, pH 9.4 |
Some smaller muscle fibers Central nuclei Other muscle fibers Central regions are basophilic. |
Abnormal internal architecture. Central dark staining. Coarse Radial strands |
Type I (light) muscle fibers Smaller than type II Clear central regions |
Muscle fibers with abnormal internal architecture, including "radial" strands and necklace fibers ![]() NADH stain |
Centronuclear myopathy: Juvenile with Necklace fibers |
![]() H&E stain |
![]() H&E stain |
Muscle fiber sizes: Bimodal distribution Central nuclei: Especially in smaller fibers Clefts: In center of other muscle fibers ![]() H&E stain |
![]() Gomori trichrome stain |
Central nuclei: Especially in smaller fibers Clefts: In center of other muscle fibers Rings (Necklaces): In some muscle fibers ![]() Gomori trichrome stain |
Necklace fibers Dark fibers: Rings Light fibers: Irregular internal architecture ![]() NADH stain |
![]() NADH stain |
![]() ![]() ATPase pH 9.4 stain |
Small muscle fibers Most are type 1 Contain central clear regions ![]() ATPase pH 4.3 stain |
![]() Esterase stain |
Centronuclear myopathy: Juvenile with many Central nuclei & Type 2C fibers |
![]() H&E stain |
Many muscle fibers have single central nuclei![]() H&E stain |
![]() VvG stain |
![]() NADH stain Abnormal internal architecture around central nuclei |
![]() ATPase pH 9.4 stain |
![]() ATPase pH 4.3 stain Many abnormal, intermediate-staining (type 2C), muscle fibers |
Centronuclear myopathy: Juvenile with Small muscle fibers & Punctate central NADH stain |
![]() H&E stain |
Many muscle fibers have single central nuclei![]() H&E stain stain |
![]() NADH stain Punctate central staining in many muscle fibers |
![]() ATPase pH 9.4 stain |
Type 1 muscle fibers are smaller than type 2![]() ATPase pH 4.3 stain |
![]() H&E stain |
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Myopathic muscle Central nuclei: Single; In most muscle fibers Endomysial connective tissue: Increased between muscle fibers Fat: Replaces perimysium and some muscle ![]() Gomori trichrome stain |
![]() H&E stain |
![]() NADH stain Abnormal internal architecture Muscle fibers may have radial strands, necklace formations or central "dots" |
![]() NADH stain |
![]() NADH stain |
![]() ATPase pH 9.4 stain |
![]() ATPase pH 4.3 stain Fiber types: All type I |
![]() Acid phosphatase stain |
![]() Cytochrome oxidase stain |
Central abnormalities in internal architecture Lysosomal (Acid phosphatase positive; Above) Mitochondrial (COX positive; Left) Endoplasmic reticulum (Caveolin-3 positive; Below) ![]() Caveolin-3 stain |
Cytoplasmic abnormalities: Dystrophin & Desmin staining ![]() Dystrophin stain |
![]() Desmin stain |
Nuclei: Large central nuclei with irregular emerin ![]() Emerin stain |
![]() H&E stain stain |
![]() H&E stain |
![]() stain |
![]() Congo red stain |
![]() H&E stain |
![]() VvG stain |
![]() VvG stain |
![]() NADH stain |
![]() NADH stain |
![]() NADH stain |
![]() NADH stain |
![]() Ultrastructure From C Cai |
![]() Ultrastructure From C Cai |
![]() Ultrastructure From C Cai |
![]() Ultrastructure From C Cai |
![]() H&E stain |
![]() Congo red stain |
![]() H&E stain |
![]() Congo red stain |
![]() H&E stain |
![]() Acid phosphatase stain |
![]() VvG stain |
![]() Gomori trichrome stain |
![]() PAS stain |
![]() SDH stain |
![]() COX stain |
![]() NADH stain |
![]() VvG stain |
![]() ATPase pH 9.4 stain |
![]() ATPase pH 4.3 stain |
![]() Esterase stain |
![]() VvG stain |
![]() H&E stain |
![]() VvG stain |
![]() H&E stain |
![]() Gomori trichrome stain |
![]() VvG stain |
![]() VvG stain |
![]() ATPase pH 9.4 stain |
![]() ATPase pH 4.3 stain |
![]() NADH stain |
![]() NADH stain |
![]() H&E stain |
![]() Gomori trichrome stain |
![]() VvG stain |
![]() VvG stain |
![]() Congo red stain |
![]() Congo red stain |
![]() VvG stain |
![]() H&E stain |
![]() H&E stain |
![]() Gomori trichrome stain |
![]() Gomori trichrome stain |
![]() VvG stain |
v![]() VvG stain |
![]() Congo red stain |
![]() VvG stain |
![]() VvG stain |
![]() VvG stain |
![]() Congo red stain |
![]() NADH stain |
![]() NADH stain |
![]() NADH stain |
![]() LAMP2 stain |
![]() LAMP2 stain |
![]() LAMP2 stain |
![]() LAMP2 stain |
![]() VvG stain |
![]() H&E stain |
![]() H&E stain |
![]() VvG stain |
![]() Congo red stain |
![]() Congo red stain |
![]() NADH stain |
![]() NADH stain |
![]() NADH stain |
![]() NADH stain |
![]() Desmin stain |
![]() COX stain |
![]() Acid phosphatase stain |
![]() ATPase ph 9.4 stain |
![]() ATPase ph 4.3 stain |