Adv Exp Med Biol
-
Glioblasotmas are the most common primary central nervous system tumor and typically have a dismal prognosis. Immunotherapy has been a promising experimental treatment. Understanding brain tumor immunobiology is critical to designing glioblasotma immunotherapies. ⋯ The antigenic underpinnings of brain tumor immunotherapy including glioma-associated and glioma-specific antigens are discussed. Finally, the molecular and cellular facets of glioma-mediated immunosuppression are outlined. The role of multiple cell types (glioma cells, glioma-infiltrating monocytes, regulatory T cells and myeloid derived suppressor cells) in mediating local and systemic immunosuppression in glioma patients is evaluated.
-
The spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases characterised by progressive lack of motor coordination leading to major disability. SCAs show high clinical, genetic, molecular and epidemiological variability. ⋯ The scope of this chapter is to provide an updated information on Machado-Joseph disease (MJD), the most frequent SCA subtype worldwide and other rare spinocerebellar ataxias including dentatorubral-pallidoluysian atrophy (DRPLA), the X-linked fragile X tremor and ataxia syndrome (FXTAS) and the nonprogressive episodic forms of inherited ataxias (EAs). Furthermore, the different therapeutic strategies that are currently being investigated to treat the ataxia and non-ataxia symptoms in SCAs are also described.