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An ultrastructural study on the cerebellum of the brindled mouse
Abstract:
Chronological morphological alterations of the cerebellum, with particular attention to the Purkinje cells, were investigated in the brindled mottled MObr mouse, a neurological mutant mouse with close clinical similarity to Kinky hair syndrome (KHS) in humans. Seven days post-natally, slight irregularity in the morphology of mitochondria of the Purkinje cell perikarya was the only significant difference between hemizygous MObr mice and litter mate controls. With advancing age the mitochondrial change became more pronounced gradually in the former, not only in the perikarya but also in the dendrites. However, by day 31 or later the mitochondrial change subsided gradually and by day 91, the mitochondria in the majority of Purkinje cells became indistinguishable from those of littermate controls. Despite the extensive mitochondrial alteration, degeneration and necrosis of Purkinje cells were rather mild. Degeneration of white matter was quite conspicuous in the mutant mouse older than 31 days. These morphological changes of the cerebellum are compared with those of other neurological mutant mice, Nervous and Purkinje cell degeneration, and with KHS in humans.
Insights
The brindled mottled MObr mouse model exhibits temporary mitochondrial alterations in Purkinje cells, with later white matter degeneration, offering insights into Kinky Hair Syndrome (KHS).
Area of Science:
- Neuroscience
- Developmental Biology
- Genetics
Background:
- The brindled mottled MObr mouse is a neurological mutant with similarities to human Kinky Hair Syndrome (KHS).
- Understanding the chronological morphological changes in this model is crucial for studying neurodegenerative disorders.
Purpose of the Study:
- To investigate the temporal morphological alterations in the cerebellum, specifically Purkinje cells, of the MObr mouse.
- To compare these changes with other neurological mutants and human KHS.
Main Methods:
- Chronological morphological examination of cerebellum and Purkinje cells in MObr mice from postnatal day 7 to 91.
- Comparative analysis with control littermates and other neurological mutant mouse models.
Main Results:
- Early (postnatal day 7) mitochondrial irregularities in Purkinje cell perikarya of MObr mice.
- Progressive mitochondrial changes in perikarya and dendrites, followed by gradual subsiding by day 91.
- Mild Purkinje cell degeneration but conspicuous white matter degeneration in older MObr mice (older than 31 days).
Conclusions:
- The MObr mouse displays transient mitochondrial abnormalities in Purkinje cells, with later white matter pathology.
- These findings contribute to understanding the pathogenesis of Kinky Hair Syndrome and related neurological disorders.