First Author | Kong J | Year | 2000 |
Journal | Neurosci Lett | Volume | 281 |
Issue | 1 | Pages | 72-4 |
PubMed ID | 10686419 | Mgi Jnum | J:60455 |
Mgi Id | MGI:1353336 | Doi | 10.1016/s0304-3940(00)00808-9 |
Citation | Kong J, et al. (2000) Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis. Neurosci Lett 281(1):72-4 |
abstractText | Mutations in superoxide dismutase 1 (SOD1) cause amyotrophic lateral sclerosis (ALS) in a subset of patients. Neurofilaments (NFs), the most abundant protein in motoneurons, may play a role in motoneuron degeneration. To investigate this role, we crossed transgenic mice expressing SOD1 mutant G93A (G93A mice) with mice overexpressing mouse neurofilament subunit H (H mice) or L (L mice). G93A mice overexpressing either NF-L or NF-H developed ALS later and survived longer than the G93A mice on a wild type background. These results illustrate a beneficial role of neurofilaments in ALS and call into question of several hypotheses regarding the role of neurofilaments in the development of ALS. |