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Mature iPSC-derived astrocytes of an ALS/FTD patient carrying the TDP43^A90V mutation display a mild reactive state and release polyP toxic to motoneurons.

ABSTRACT: Astrocytes play a critical role in the maintenance of a healthy central nervous system and astrocyte dysfunction has been implicated in various neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). There is compelling evidence that mouse and human ALS and ALS/FTD astrocytes can reduce the number of healthy wild-type motoneurons (MNs) in co-cultures or after treatment with astrocyte conditioned media (ACM), independently of their genotype. A growing number of studies have shown that soluble toxic factor(s) in the ACM cause non-cell autonomous MN death, including our recent identification of inorganic polyphosphate (polyP) that is excessively released from mouse primary astrocytes (SOD1, TARDBP, and C9ORF72) and hum

SUBMITTER: Rojas F

PROVIDER: S-EPMC10461635 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature

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Dataset Information

Mature iPSC-derived astrocytes of an ALS/FTD patient carrying the TDP43^A90V mutation display a mild reactive state and release polyP toxic to motoneurons.

OmicsDI is part of the ELIXIR infrastructure

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Dataset Information

Mature iPSC-derived astrocytes of an ALS/FTD patient carrying the TDP43A90V mutation display a mild reactive state and release polyP toxic to motoneurons.

OmicsDI is part of the ELIXIR infrastructure

Tweets

Mature iPSC-derived astrocytes of an ALS/FTD patient carrying the TDP43^A90V mutation display a mild reactive state and release polyP toxic to motoneurons.