Huntington's disease is an autosomal-dominant, progressive neurodegenerative disorder
with a distinct phenotype, including chorea and dystonia, incoordination, cognitive decline …

Recent studies undoubtedly show the importance of inter organellar connections to maintain
cellular homeostasis. In normal physiological conditions or in the presence of cellular and …

An expansion of a trinucleotide CAG repeat on chromosome 4 causes Huntington disease.
The abnormal elongation of the CAG increases the polyglutamine stretch of huntingtin …

Huntington's disease (HD) is an autosomal dominant disorder characterized by
abnormalities of movement, cognition, and emotion and selective atrophy of the striatum and …

Huntington's disease (HD) is one of the most common autosomal dominant inherited,
neurodegenerative disorders. It is characterized by progressive motor, emotional and …

Publisher Summary This chapter emphasizes that premature death of medium spiny
GABAergic, projection neurons of the neostriatum with gradual atrophy of the caudate …

Abstract Huntington disease (HD)-like 2 (HDL2) is a rare genetic disease caused by an
expanded trinucleotide repeat in the JPH3 gene (encoding junctophilin 3) that shows …

The unfolded protein response (UPR) plays a vital role in maintaining cell homeostasis as a
consequence of endoplasmic reticulum (ER) stress. However, prolonged UPR activity leads …

About 1% of suspected Huntington's disease cases emerge as phenocopy syndromes. Such
syndromes are clinically important in their own right but may also shed light on the …

Abstract Huntington's disease (HD), which is caused by a triplet-repeat expansion in the
IT15 gene (also known as huntingtin or HD), accounts for about 90% of cases of chorea of …