Topic: spastin

Choroid plexus (ChP) is located in cerebral ventricles, where it produces cerebrospinal fluid (CSF), forms the selective blood-CSF barrier and controls the homeostasis of the central nervous system. ChP is primarily composed of a monolayer of specialized epithelial cells producing cerebrospinal fluid. Differentiated ChP epithelial cells harbor multiple cilia on the CSF-facing surface, but their development and function are still controversial. We have delineated the sequential progression of cilia formation in the ChP epithelial cells by exploiting super-resolution microscopy techniques and mo

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