The dominantly inherited spinocerebellar ataxias (SCAs) are a large and diverse group of
neurodegenerative diseases. The most prevalent SCAs (SCA1, SCA2, SCA3, SCA6 and …

Cerebellar ataxias with autosomal dominant transmission are rare, but identification of the
associated genes has provided insight into the mechanisms that could underlie other forms …

Abstract Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant disorder
characterized by neurodegeneration of the cerebellum, spinal cord and brainstem. A 1.2 …

The autosomal dominant cerebellar ataxias (ADCAs) represent a heterogeneous group of
neurodegenerative diseases with progressive ataxia and cerebellar degeneration. The …

A growing number of neurodegenerative diseases have been found to result from the
expansion of an unstable trinucleotide repeat. Over the past 6 years, researchers have …

Polyglutamine (polyQ) diseases are a group of inherited neurodegenerative disorders
caused by expanded cytosine-adenine-guanine (CAG) repeats encoding proteins with …

The aim of the present study was (i) to compare disease progression and survival in different
types of degenerative ataxia, and (ii) to identify variables that may modify the rate of disease …

Seventy‐seven families with autosomal dominant cerebellar ataxia were analyzed for the
CAG repeat expansions causing spinocerebellar ataxia (SCA) types 1, 2, 3, and 6. The …

Within the closing decade of the twentieth century, 14 neurological disorders were shown to
result from the expansion of unstable trinucleotide repeats, establishing this once unique …

Abstract Spinocerebellar ataxia type 6 (SCA6) was recently identified as a form of autosomal
dominant cerebellar ataxia associated with small expansions of the trinucleotide repeat …