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Fig. 7. Degeneration and pathological cytoplasmic aggregates in mutant neurones,
and abnormality of mutant ependyma and choroid plexi. (A-D) Degenerating
neurones. (A) Semithin section stained with Toluidine Blue showing abundant
shrunken and hyperchromic degenerating cells in the thalamus (centromedian
nucleus) of mutant mice. The same cell abnormalities were observed in dorsal
striatum and septum (not shown). (B) Detail of A. White asterisks indicate
healthy neurones; black asterisks indicate shrunken and hyperchromatic
pathological neurones. (C,D) Electron microscopy of hyperchromatic cell. Note
the high electron density of the cytoplasm and nucleus, the cytoplasmic and
nuclear membrane irregularities, and the dilated Golgi cisternae (indicated by
arrows). N, nucleus; M, mitochondria. Scale bars: 2.5 µm in C; 0.5 µm in
D. (E-L) Intraneuronal aggregates. (E) Semithin section stained with Toluidine
Blue showing abundant intraneuronal perinuclear aggregates in the thalamus
(ventrobasal nuclei) of mutant mice. The inclusions in the cells at the far
left and lower right are similar to those shown by electron microscopy in F
and G, respectively. Arrowheads point to aggregates. V, blood vessel. (F,G,H)
Electron microscopy of aggregates. Asterisks indicate the central clearer
region, seen at higher magnification in H. The inclusions show abundant
filamentous content, with a filament diameter of 9-10 µm. Scale bars: 1
µm in F,G; 0.2 µm in H. (I) Semithin section showing
inclusion-containing and normal neurones, stained with Toluidine Blue. Arrow
indicates aggregate showing typical perinuclear location. (J-L)
Immunohistochemistry (on paraffin sections) with anti-neurofilament (SMI 32;
J) and anti-ubiquitin (K,L) antibodies. Positive staining was also observed
with SMI 31 (anti-phosphorylated neurofilaments) antibody (not shown). Arrows
indicate immunostained intraneuronal aggregates. Note the normal morphology of
the aggregate-containing neurones in J and K, and the pathological morphology
of the hyperchromatic aggregate-containing neurone in L (see text). In K, note
the weaker immunostaining (light brown) in the cell on the right. (M,N)
Wild-type (M) and mutant (N) ependyma. Black diamond indicates lipidic
inclusions; arrows indicate cilia. N, nucleus. (O,P) Wild-type (O) and mutant
(P) choroid plexi. Left, apical microvilli; right, basal membrane, with
numerous infoldings (asterisk) in the mutant. Arrowheads point to basal
membrane.
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