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Nemaline myopathy rod bodies. Structure and composition.

M Yamaguchi, R M Robson, M H Stromer

    Journal of the Neurological Sciences
    |October 1, 1982
    PubMed
    Summary

    Calcium-activated protease (CAF) digestion revealed that nemaline rods are primarily composed of actin filaments, similar to Z-lines. This protease treatment also suggests alpha-actinin

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    Area of Science:

    • Muscle ultrastructure
    • Protein biochemistry
    • Cellular biology

    Background:

    • Nemaline myopathy is characterized by the presence of abnormal protein aggregates known as nemaline rods.
    • The precise composition and structure of nemaline rods and their relationship to Z-lines remain incompletely understood.
    • Understanding these structures is crucial for elucidating the pathophysiology of nemaline myopathy.

    Purpose of the Study:

    • To elucidate the ultrastructural composition of nemaline rods in nemaline myopathy.
    • To investigate the relationship between nemaline rods and Z-lines.
    • To identify the key protein components of nemaline rods.

    Main Methods:

    • Glycerinated nemaline myopathy muscle was treated with calcium-activated protease (CAF).

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  • Electron microscopy was used to examine the structural changes and identify filament arrangements.
  • Heavy meromyosin (HMM) labeling was employed to identify actin filaments.
  • Gel electrophoresis was performed on CAF-treated muscle to analyze protein composition.

    • Main Results:

    • CAF digestion exposed 6-7 nm backbone filaments within nemaline rods, identified as actin filaments via HMM decoration.
    • CAF treatment removed cross-striated patterns and Z-filament-like connections, suggesting alpha-actinin's role in rod structure.
    • Gel electrophoresis revealed that alpha-actinin was largely released, with actin and myosin remaining in the residue.
    • Intact rods exhibited oblique filament patterns, thin (7 nm) and thick (11 nm) lines, and amorphous material, varying with sectioning.
    • Cross-sections showed predominantly small square net (SS) and basket-weave (BW) forms, also present in Z-lines.

    Conclusions:

    • Nemaline rods are lateral polymers of Z-line units, primarily composed of actin filaments.
    • Alpha-actinin likely plays a role in the formation and structural organization of nemaline rods.
    • Rod length and Z-line width may be determined by the overlap of oppositely polarized actin filaments.
    • Deregulation of actin filament length could initiate nemaline rod formation.