Hereditary hypotrophic axonopathy with neurofilament deficiency in a mutant strain of the Japanese quail

@article{Yamasaki2004HereditaryHA,
  title={Hereditary hypotrophic axonopathy with neurofilament deficiency in a mutant strain of the Japanese quail},
  author={H. Yamasaki and Chitoshi Itakura and Makoto Mizutani},
  journal={Acta Neuropathologica},
  year={2004},
  volume={82},
  pages={427-434}
}
SummaryA primary axonal disease affecting the central and peripheral nervous system was discovered in a new mutant strain of the Japanese quail, named quiver (Quv). Quv showed significantly smaller cross-sectional areas of the cervical spinal cord and the optic and sciatic nerves, when compared with controls by light microscopic morphometry. In the cervical spinal cord, electron microscopic morphometry indicated that myelinated axons in Quv were significantly smaller in size than in controls… 
Smaller number of large myelinated fibers and focal myelin thickening in mutant quails deficient in neurofilaments
TLDR
It is concluded that a smaller number of large myelination fibers with a greater number of small myelinated fibers and the presence of focal myelin thickening are the main morphologic findings in this mutant, probably due to the arrest of radial growth or maturation of the axons of the myelinate fibers in the absence of ongoing myelinating fiber degeneration.
Acrylamide-induced peripheral neuropathy in normal and neurofilament-deficient Japanese quails
TLDR
It is demonstrated that NF-deficient Quv quails are sensitive to neurotoxic effects of AC, and the different pathology of AC-intoxicated normal and Quv qualis indicates the presence or absence of NFs influences the appearance and extent of AC axonopathy.
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Knockout Models of Neurofilament Proteins
TLDR
How mouse knockout models of NF proteins have been used to study the multiple aspects of NF biology is described.
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TLDR
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Neuronal intermediate filaments consist of the NFL subunit linked with NFM and NFH, and their alterations have been proposed as a pathogenesic cause in motor neuron diseases. Depletion of the Nefl
Smaller axon and unaltered numbers of microtubules per axon in relation to number of myelin lamellae of myelinated fibers in the mutant quail deficient in neurofilaments
TLDR
Findings indicate that there was a less significant effect of NF deficiency on the smaller than on the larger myelinated fibers in the mutant quails deficient in neurofilaments.
Greater number of microtubules per axon of unmyelinated fibers of mutant quails deficient in neurofilaments: possible compensation for the absence of neurofilaments
TLDR
A significantly greater number of MT per axon in the mutants may suggest a compensatory increase of MT in the absence of NF, which may conserve the size and transverse circular profile of the unmyelinated axons which are probably maintained by both MT and NF in the controls.
Acrylamide-Induced Changes in the Neurofilament Protein of Rat Cerebrum Fractions
TLDR
The present results suggested that the change of NF-L and NF-M levels in cerebrum might be relevant to the mechanisms of the neurofilamentous axonopathies induced by ACR.
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