Since the identification of the causative gene in Huntington's disease (HD), a number of
animal models of this disorder have been developed. A frequently asked question is: which …

The basal ganglia are a highly interconnected set of subcortical nuclei and major atrophy in
one or more regions may have major effects on other regions of the brain. Therefore, the …

NAD metabolism regulates diverse biological processes, including ageing, circadian rhythm
and axon survival. Axons depend on the activity of the central enzyme in NAD biosynthesis …

Huntington's disease (HD) is an inherited neurodegenerative disorder caused by a
polyglutamine stretch in the huntingtin protein. Today, more than 15 years after the genetic …

Spinal muscular atrophy (SMA) is a common pediatric neuromuscular disorder caused by
insufficient levels of the survival of motor neuron (SMN) protein. Studies involving SMA …

Expanded CAG repeats lead to debilitating neurodegenerative disorders characterized by
aggregation of proteins with expanded polyglutamine (polyQ) tracts. The mechanism of …

Huntington's disease is a genetic neurodegenerative disorder with symptoms that are linked
to the progressive dysfunction and neuronal death in corticostriatal circuits. The causative …

We investigated the role of PPAR γ coactivator 1α (PGC-1α) in muscle dysfunction in
Huntington's disease (HD). We observed reduced PGC-1α and target genes expression in …

Recent developments in our understanding of the pathophysiological mechanisms
underlying degeneration in both the central and peripheral nervous systems have …

White matter abnormalities are a nearly universal pathological feature of neurodegenerative
disorders including Huntington disease (HD). A long-held assumption is that this white …