Deletion of Transferrin Receptor 1 in Parvalbumin Interneurons Induces a Hereditary Spastic Paraplegia-Like Phenotype.

ABSTRACT: Hereditary spastic paraplegia (HSP) is a severe neurodegenerative movement disorder, the underlying pathophysiology of which remains poorly understood. Mounting evidence has suggested that iron homeostasis dysregulation can lead to motor function impairment. However, whether deficits in iron homeostasis are involved in the pathophysiology of HSP remains unknown. To address this knowledge gap, we focused on parvalbumin-positive (PV⁺) interneurons, a large category of inhibitory neurons in the central nervous system, which play a critical role in motor regulation. The PV⁺ interneuron-specific deletion of the gene encoding transferrin receptor 1 (TFR1), a key component of the neuronal iron uptake machinery, induced severe progressive motor deficits in both male and femal

SUBMITTER: Xiong W 

PROVIDER: S-EPMC10325000 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature