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

@article{citeulike:5016975, 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.}, author = {Xu, Z. and Cork, L. C. and Griffin, J. W. and Cleveland, D. W.}, citeulike-article-id = {5016975}, citeulike-linkout-0 = {http://view.ncbi.nlm.nih.gov/pubmed/8462100}, citeulike-linkout-1 = {http://www.hubmed.org/display.cgi?uids=8462100}, issn = {0092-8674}, journal = {Cell}, keywords = {als, mnd, model, mouse, neurofilament}, month = {April}, number = {1}, pages = {23--33}, posted-at = {2009-06-30 01:25:34}, priority = {2}, title = {Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease.}, url = {http://view.ncbi.nlm.nih.gov/pubmed/8462100}, volume = {73}, year = {1993} }

TY - JOUR ID - citeulike:5016975 L3 - citeulike-article-id:5016975 N2 - 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. IS - 1 JF - Cell SN - 0092-8674 EP - 33 TI - Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease. VL - 73 SP - 23 KW - als KW - mnd KW - model KW - mouse KW - neurofilament AU - Xu, Z AU - Cork, LC AU - Griffin, JW AU - Cleveland, DW PY - 1993/04/09/ UR - http://view.ncbi.nlm.nih.gov/pubmed/8462100 ER -