Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
Cell. 1993 Apr 9;73(1):23-33.

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

Author information

  • 1Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205.

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.

PMID:
8462100
[PubMed - indexed for MEDLINE]

LinkOut - more resources

Full Text Sources

Other Literature Sources

Molecular Biology Databases

Miscellaneous

PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science
    Loading ...
    Write to the Help Desk