Topic: microtubule-severing protein

Hereditary Spastic Paraplegia 4 (HSP-SPG4) is a rare neurodegenerative disease characterized by degeneration of the corticospinal tract caused by mutations of the SPAST gene encoding Spastin, a microtubule-severing protein. We recently showed that gain-of-function cytotoxicity of the M1 isoform of Spastin is sufficient to account for the disease. As a result of the toxic properties of mutant-M1, microtubules in the corticospinal tracts become less acetylated, while the activity of Histone Deacetylates 6 (HDAC6) is correspondingly increased. In addition, it has been reported that Tau, a regulat