The spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of autosomal
dominantly inherited progressive disorders, the clinical hallmark of which is loss of balance …

The spinocerebellar ataxias type 1 (SCA1), 2 (SCA2), 3 (SCA3), 6 (SCA6) and 7 (SCA7) are
genetically defined autosomal dominantly inherited progressive cerebellar ataxias (ADCAs) …

Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine
(polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective …

Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA 3), is an incurable disorder, widely regarded as the most common form of …

Mitochondria have been largely described as the powerhouse of the cell and recent findings
demonstrate that this organelle is fundamental for neurogenesis. The mechanisms …

Intensive scientific research devoted in the recent years to understand the molecular
mechanisms or neurodegeneration in spinocerebellar ataxias (SCAs) are identifying new …

Several morphological changes, centered in/around Purkinje cells (PCs), have been
identified in the cerebellum of essential tremor (ET) patients. These changes have not been …

Spinocerebellar ataxia type 3, spinocerebellar ataxia type 6 and Friedreich's ataxia are
common hereditary ataxias. Different patterns of atrophy of the cerebellar cortex are well …

Spinocerebellar ataxias (SCAs) are devastating neurodegenerative disorders for which no
curative or preventive therapies are available. Deregulation of brain cholesterol metabolism …

Recent advances in understanding dominant spinocerebellar ataxias from clinical and
genetic points of view - PMC Back to Top Skip to main content NIH NLM Logo Access keys …