Stem Cells · 2018 · 95 citations · 57 references
Life SciencesNeurologyNeuroscienceCellular NeuroscienceGlial BiologyNeurodegenerationAmyotrophic Lateral SclerosisGlial Cell BiologyNeural Stem CellsNeurodegenerative DiseasesNeurodegenerative DisordersNeurotrophic FactorsAmyotrophic Lateral Sclerosis ModelsAmyotrophic Lateral Sclerosis PathologyNeural Stem Cell TransplantationNeurotrophic Factor ReleaseNeurotrophic Factor SignalingNeurotrophic Support In DiseaseProgenitor CellsSod1 Pathology
Abstract Early dysfunction of cortical motor neurons may underlie the initiation of amyotrophic lateral sclerosis (ALS). As such, the cortex represents a critical area of ALS research and a promising therapeutic target. In the current study, human cortical-derived neural progenitor cells engineered to secrete glial cell line-derived neurotrophic factor (GDNF) were transplanted into the SOD1G93A ALS rat cortex, where they migrated, matured into astrocytes, and released GDNF. This protected motor neurons, delayed disease pathology and extended survival of the animals. These same cells injected into the cortex of cynomolgus macaques survived and showed robust GDNF expression without adverse effects. Together this data suggests that introducing cortical astrocytes releasing GDNF represents a novel promising approach to treating ALS.
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