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Deficiency of Perry syndrome-associated p150Glued in midbrain dopaminergic neurons leads to progressive neurodegeneration and endoplasmic reticulum abnormalities

6 Mar 2023

Abstract excerpt

Abstract Multiple missense mutations in p150 Glued are linked to Perry syndrome (PS), a rare neurodegenerative disease pathologically characterized by loss of nigral dopaminergic (DAergic) neurons. Here we generated p150 Glued conditional knockout (cKO) mice by deleting p150 Glued in midbrain DAergic neurons. The young cKO mice displayed impaired motor coordination, dystrophic DAergic dendrites, swollen axon...

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PMID: 36879021
DOI: 10.1038/s41531-023-00478-0

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