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Washington University Experience | MUSCLE | Nemaline rod myopathy | 4B4A (Case 4) EM053 copy - Copy

4B4A-C In this case there are areas of palisaded Z lines (arrowheads) as well as adjacent disorganized nemaline-like rods (arrow) as shown at higher magnification in images #4B4B,C). The question in these cases involves separation of genuine nemaline rods from disorganized sarcomeres with displaced Z-line material. It has been suggested that they are separable based on their dimensions, anatomical location, and impact on sarcomere organization. However, since they share an identical internal protein lattice-like structure, reflecting their origin from Z-lines, separation may be problematic. The designation of “nemaline-like” rods may be warranted but is not satisfying. Often both structures are present as might be expected if Z-lines give rise to nemaline rods.

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