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Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease.

Abstract：

:Excessive accumulation of neurofilaments in the cell bodies and proximal axons of motor neurons is a major pathological hallmark during the early stages of many human motor neuron diseases. To test directly the consequence of overexpression of the major neurofilament subunit NF-L, we produced transgenic mice that accumulate NF-L to approximately 4-fold the normal level in the sciatic nerve. In young animals, the motor neurons of the ventral horn of the spinal cord have massive accumulations of neurofilaments, swollen perikarya, and eccentrically localized nuclei. NF-L accumulation is accompanied by an increased frequency of axonal degeneration, proximal axon swelling, and severe skeletal muscle atrophy. These data indicate that extensive accumulation of neurofilaments in motor neurons can trigger the neurodegenerative process.

journal_name

Cell

journal_title

Cell

authors

Xu Z,Cork LC,Griffin JW,Cleveland DW

doi

10.1016/0092-8674(93)90157-l

subject

Has Abstract

pub_date

1993-04-09 00:00:00

pages

23-33

issue

1

eissn

0092-8674

issn

1097-4172

pii

0092-8674(93)90157-L

journal_volume

73

pub_type

杂志文章

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