Spastin Is Required to Prevent SPAST-Related Demyelination.

Abstract excerpt

Mutations in the SPAST gene, encoding the microtubule-severing protein Spastin, cause the most common type of hereditary spastic paraplegia (HSP): SPG4, a disorder primarily characterized by length-dependent axonal degeneration. Clinically, most SPG4 patients present with a pure phenotype marked by progressive spasticity in the lower extremities. It has also been reported that complex cases exhibit demyelination...

Topics

Join the communities discussing this publication.

• Coculture Techniques
• Demyelinating Diseases
• Mice, Inbred C57BL
• Myelin Sheath
• Neurons