Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities
Huntington disease (HD) is a neurodegenerative disease caused by CAG repeat expansion
in the huntingtin gene (HTT) and involves a complex web of pathogenic mechanisms …
in the huntingtin gene (HTT) and involves a complex web of pathogenic mechanisms …
The biology of huntingtin
F Saudou, S Humbert - Neuron, 2016 - cell.com
Huntingtin (HTT) is now a famous protein because an abnormal expansion of a glutamine
stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …
stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …
[HTML][HTML] CAG repeat not polyglutamine length determines timing of Huntington's disease onset
Variable, glutamine-encoding, CAA interruptions indicate that a property of the uninterrupted
HTT CAG repeat sequence, distinct from the length of huntingtin's polyglutamine segment …
HTT CAG repeat sequence, distinct from the length of huntingtin's polyglutamine segment …
Molecular mechanisms and potential therapeutical targets in Huntington's disease
C Zuccato, M Valenza, E Cattaneo - Physiological reviews, 2010 - journals.physiology.org
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG repeat
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …
Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice
The precise cause of neuronal death in Huntington's disease (HD) is unknown. Although no
single specific protein-protein interaction of mutant huntingtin has emerged as the …
single specific protein-protein interaction of mutant huntingtin has emerged as the …
The hunt for huntingtin function: interaction partners tell many different stories
P Harjes, EE Wanker - Trends in biochemical sciences, 2003 - cell.com
Huntington's disease (HD) is a neurodegenerative disorder caused by an abnormally
elongated polyglutamine (polyQ) tract in the large protein huntingtin (htt). Currently, both the …
elongated polyglutamine (polyQ) tract in the large protein huntingtin (htt). Currently, both the …
Huntingtin–protein interactions and the pathogenesis of Huntington's disease
SH Li, XJ Li - TRENDS in Genetics, 2004 - cell.com
At least nine inherited neurodegenerative diseases share a polyglutamine expansion in
their respective disease proteins. These diseases show distinct neuropathological changes …
their respective disease proteins. These diseases show distinct neuropathological changes …
Huntingtin and the molecular pathogenesis of Huntington's disease: Fourth in Molecular Medicine Review Series
Huntington's disease (HD) is a late-onset neurodegenerative disorder that is caused by a
CAG repeat expansion in the IT15 gene, which results in a long stretch of polyglutamine …
CAG repeat expansion in the IT15 gene, which results in a long stretch of polyglutamine …
Transcriptional abnormalities in Huntington disease
KL Sugars, DC Rubinsztein - TRENDS in Genetics, 2003 - cell.com
Huntington disease (HD) is caused by a CAG repeat expansion that is translated into an
abnormally long polyglutamine (polyQ) tract in the huntingtin protein. The precise …
abnormally long polyglutamine (polyQ) tract in the huntingtin protein. The precise …
Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons
JA Parker, M Arango, S Abderrahmane, E Lambert… - Nature …, 2005 - nature.com
We report that Sir2 activation through increased sir-2.1 dosage or treatment with the sirtuin
activator resveratrol specifically rescued early neuronal dysfunction phenotypes induced by …
activator resveratrol specifically rescued early neuronal dysfunction phenotypes induced by …