Title : Striatal neuronal death mediated by astrocytes from the Gcdh-/- mouse model of glutaric acidemia type I.

Pub. Date : 2015 Aug 15

PMID : 25968119






3 Functional Relationships(s)
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1 Upon Lys treatment, Gcdh-/- astrocytes synthetized and released GA and 3-hydroxyglutaric acid (3HGA). glutaric acid glutaryl-Coenzyme A dehydrogenase Mus musculus
2 In contrast, Lys or GA direct exposure on Gcdh-/- or WT striatal neurons cultured in the absence of astrocytes was not toxic, indicating that neuronal death is mediated by astrocytes. glutaric acid glutaryl-Coenzyme A dehydrogenase Mus musculus
3 In summary, GCDH-defective astrocytes actively contribute to produce and accumulate GA and 3HGA when Lys catabolism is stressed. glutaric acid glutaryl-Coenzyme A dehydrogenase Mus musculus