Cerebellar reserve refers to the capacity of the cerebellum to compensate for tissue damage
or loss of function resulting from many different etiologies. When the inciting event produces …

Immune-mediated cerebellar ataxias (IMCAs), a clinical entity reported for the first time in the
1980s, include gluten ataxia (GA), paraneoplastic cerebellar degenerations (PCDs) …

Alterations in Purkinje neuron firing often accompany ataxia, but the molecular basis for
these changes is poorly understood. In a mouse model of spinocerebellar ataxia type 2 …

CAPRIN1 is a ubiquitously expressed protein, abundant in the brain, where it regulates the
transport and translation of mRNAs of genes involved in synaptic plasticity. Here we …

Dysfunction of neuronal circuits is an important determinant of neurodegenerative diseases.
Synaptic dysfunction, death, and intrinsic activity of neurons are thought to contribute to the …

Spinocerebellar ataxias (SCAs) are a heterogeneous group of dominantly inherited
neurodegenerative disorders affecting the cerebellum and its associated pathways. There …

Induced pluripotent stem cell (iPSC) technology has emerged as an important tool in
understanding, and potentially reversing, disease pathology. This is particularly true in the …

The cerebellum is a vulnerable target of autoimmunity in the CNS. The category of immune-
mediated cerebellar ataxias (IMCAs) was recently established, and includes in particular …

Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by
expansions of a polyglutamine (polyQ) tract in the androgen receptor (AR) gene. SBMA is …

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant ataxia caused by an
expansion of CAG repeats in the exon 1 of the gene ATXN2, conferring a gain of toxic …