RGD Reference Report - Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis. - Rat Genome Database

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Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis.

Authors: Kong, J  Xu, Z 
Citation: Kong J and Xu Z, Neurosci Lett. 2000 Mar 3;281(1):72-4.
RGD ID: 13525000
Pubmed: PMID:10686419   (View Abstract at PubMed)

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.




  
Object Symbol
Species
Term
Qualifier
Evidence
With
Notes
Source
Original Reference(s)
NEFHHumanamyotrophic lateral sclerosis treatmentISORGD:10969 RGD 
NEFLHumanamyotrophic lateral sclerosis treatmentISORGD:1552280 RGD 
NefhRatamyotrophic lateral sclerosis treatmentISORGD:10969 RGD 
NefhMouseamyotrophic lateral sclerosis treatmentIDA  RGD 
NeflRatamyotrophic lateral sclerosis treatmentISORGD:1552280 RGD 
NeflMouseamyotrophic lateral sclerosis treatmentIDA  RGD 


Genes (Rattus norvegicus)
Nefh  (neurofilament heavy chain) Nefl  (neurofilament light chain)

Genes (Mus musculus)
Nefh  (neurofilament, heavy polypeptide) Nefl  (neurofilament, light polypeptide)

Genes (Homo sapiens)
NEFH  (neurofilament heavy chain) NEFL  (neurofilament light chain)