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. 2006 Mar 21;103(13):5137–5142. doi: 10.1073/pnas.0601082103

Fig. 4.

Fig. 4.

Organelle accumulation and axonal degeneration in NCP1 and CGT mutants. (A) Section of a WT axon through the paranodal region (arrows) showing normal axonal diameter and cytoskeletal organization. (B) An NCP1 mutant axon shows a large swelling at the paranodal region (arrows). Note the accumulation of mitochondria (arrowheads) and cytoskeletal disorganization. (C) A CGT mutant axon also shows accumulation of mitochondria (arrowheads). Note the axon diameter disparity between WT and mutants. (D–E) In NCP1 mutant axons, the most abundant components of the swellings are mitochondria and SER. Accumulation of SER results in membrane lattices, which fill most of the swellings (arrowheads). (F) The CGT mutant axonal swellings also develop SER membrane lattices as in NCP1 mutants (arrowheads). (G–H) NCP1 mutant axons in the process of degeneration showing vacuolation (arrowheads). (I) A CGT mutant axon in an early stage of degeneration displays electron dense bodies (arrowheads), which are a common feature associated with axonal decay and disorganized cytoskeleton. Arrow points to a normal myelinated axon. (Scale bars: A–F, 2 μm; G–I, 1 μm.)