Unknown

Dataset Information

0

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis.


ABSTRACT: Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease that is characterized by the loss of motor neurons. Using mice carrying a deletable mutant gene, diminished mutant expression in astrocytes did not affect onset, but delayed microglial activation and sharply slowed later disease progression. These findings demonstrate that mutant astrocytes are viable targets for therapies for slowing the progression of non-cell autonomous killing of motor neurons in ALS.

SUBMITTER: Yamanaka K 

PROVIDER: S-EPMC3137510 | biostudies-literature | 2008 Mar

REPOSITORIES: biostudies-literature

altmetric image

Publications

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis.

Yamanaka Koji K   Chun Seung Joo SJ   Boillee Severine S   Fujimori-Tonou Noriko N   Yamashita Hirofumi H   Gutmann David H DH   Takahashi Ryosuke R   Misawa Hidemi H   Cleveland Don W DW  

Nature neuroscience 20080203 3


Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease that is characterized by the loss of motor neurons. Using mice carrying a deletable mutant gene, diminished mutant expression in astrocytes did not affect onset, but delayed microglial activation and sharply slowed later disease progression. These findings demonstrate that mutant astrocytes are viable targets for therapies for slowing the progression of non-cell autonomo  ...[more]

Similar Datasets

| S-EPMC4305209 | biostudies-other
| S-EPMC8069893 | biostudies-literature
| S-EPMC3207668 | biostudies-literature
| S-EPMC4137497 | biostudies-literature
| S-EPMC4404434 | biostudies-literature
| S-EPMC4940876 | biostudies-literature
| S-EPMC2538879 | biostudies-other
| S-EPMC6626434 | biostudies-other
| S-EPMC3097891 | biostudies-other
| S-EPMC9374680 | biostudies-literature