Article
Pluripotent Stem Cells as a Preclinical Cellular Model for Studying Hereditary Spastic Paraplegias.
International journal of molecular sciences - 23 Feb 2024
Damiani Devid, Baggiani Matteo, Della Vecchia Stefania, Naef Valentina, Santorelli Filippo Maria
Abstract excerpt
Hereditary spastic paraplegias (HSPs) comprise a family of degenerative diseases mostly hitting descending axons of corticospinal neurons. Depending on the gene and mutation involved, the disease could present as a pure form with limb spasticity, or a complex form associated with cerebellar and/or cortical signs such as ataxia, dysarthria, epilepsy, and intellectual disability. The progressive nature of HSPs...
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