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 …
Post-translational modifications: regulators of neurodegenerative proteinopathies
One of the hallmark features in the neurodegenerative disorders (NDDs) is the accumulation
of aggregated and/or non-functional protein in the cellular milieu. Post-translational …
of aggregated and/or non-functional protein in the cellular milieu. Post-translational …
The cryo-electron microscopy structure of huntingtin
Huntingtin (HTT) is a large (348 kDa) protein that is essential for embryonic development
and is involved in diverse cellular activities such as vesicular transport, endocytosis …
and is involved in diverse cellular activities such as vesicular transport, endocytosis …
Huntingtin structure is orchestrated by HAP40 and shows a polyglutamine expansion-specific interaction with exon 1
Huntington's disease results from expansion of a glutamine-coding CAG tract in the
huntingtin (HTT) gene, producing an aberrantly functioning form of HTT. Both wildtype and …
huntingtin (HTT) gene, producing an aberrantly functioning form of HTT. Both wildtype and …
[HTML][HTML] Targeting the proteostasis network in Huntington's disease
TR Soares, SD Reis, BR Pinho, MR Duchen… - Ageing Research …, 2019 - Elsevier
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by
a polyglutamine expansion mutation in the huntingtin protein. Expansions above 40 …
a polyglutamine expansion mutation in the huntingtin protein. Expansions above 40 …
N-terminal Huntingtin (Htt) phosphorylation is a molecular switch regulating Htt aggregation, helical conformation, internalization, and nuclear targeting
Huntington's disease is a fatal neurodegenerative disorder resulting from a CAG repeat
expansion in the first exon of the gene encoding the Huntingtin protein (Htt) …
expansion in the first exon of the gene encoding the Huntingtin protein (Htt) …
Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities
RJ Harding, Y Tong - Acta Pharmacologica Sinica, 2018 - nature.com
Many neurodegenerative diseases are characterized by impairment of protein quality control
mechanisms in neuronal cells. Ineffective clearance of misfolded proteins by the …
mechanisms in neuronal cells. Ineffective clearance of misfolded proteins by the …
Traffic signaling: new functions of huntingtin and axonal transport in neurological disease
H Vitet, V Brandt, F Saudou - Current Opinion in Neurobiology, 2020 - Elsevier
Highlights•Subtle changes in axonal transport can modulate protein accumulation at the
synapse.•The huntingtin mutation underlying Huntington's disease disrupts axonal …
synapse.•The huntingtin mutation underlying Huntington's disease disrupts axonal …
Interaction of huntingtin with PRMTs and its subsequent arginine methylation affects HTT solubility, phase transition behavior and neuronal toxicity
Huntington's disease (HD) is an incurable neurodegenerative disorder caused by a CAG
expansion in the huntingtin gene (HTT). Post-translational modifications of huntingtin protein …
expansion in the huntingtin gene (HTT). Post-translational modifications of huntingtin protein …
How do post-translational modifications influence the pathomechanistic landscape of Huntington's disease? A comprehensive review
Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disorder
characterized by the loss of motor control and cognitive ability, which eventually leads to …
characterized by the loss of motor control and cognitive ability, which eventually leads to …