Huntingtin co-isolates with small extracellular vesicles from blood plasma of TgHD and KI-HD pig models of Huntington's disease and human blood plasma

H Ananbeh, J Novak, S Juhas, J Juhasova… - International journal of …, 2022 - mdpi.com
(1) Background: Huntington's disease (HD) is rare incurable hereditary neurodegenerative
disorder caused by CAG repeat expansion in the gene coding for the protein huntingtin …

Emerging roles of exosomes in Huntington's disease

H Ananbeh, P Vodicka… - International journal of …, 2021 - mdpi.com
Huntington's disease (HD) is a rare hereditary autosomal dominant neurodegenerative
disorder, which is caused by expression of mutant huntingtin protein (mHTT) with an …

Quantification assays for total and polyglutamine-expanded huntingtin proteins

D Macdonald, MA Tessari, I Boogaard, M Smith, K Pulli… - PloS one, 2014 - journals.plos.org
The expansion of a CAG trinucleotide repeat in the huntingtin gene, which produces
huntingtin protein with an expanded polyglutamine tract, is the cause of Huntington's …

Soluble mutant huntingtin drives early human pathogenesis in Huntington's disease

A Miguez, C Gomis, C Vila… - Cellular and Molecular …, 2023 - Springer
Huntington's disease (HD) is an incurable inherited brain disorder characterised by massive
degeneration of striatal neurons, which correlates with abnormal accumulation of misfolded …

Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm

E Romero, GH Cha, P Verstreken, CV Ly, RE Hughes… - Neuron, 2008 - cell.com
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by
expansion of a translated CAG repeat in the N terminus of the huntingtin (htt) protein. Here …

[HTML][HTML] Aberrant splicing of mutant huntingtin in Huntington's disease knock-in pigs

H Tong, T Yang, L Liu, C Li, Y Sun, Q Jia, Y Qin… - Neurobiology of …, 2023 - Elsevier
Huntington's disease (HD) is an autosomal-dominant inherited neurodegenerative disease
caused by a CAG repeat expansion in exon1 of the huntingtin gene (HTT). This expansion …

Comparison of huntingtin proteolytic fragments in human lymphoblast cell lines and human brain

T Toneff, L Mende‐Mueller, Y Wu… - Journal of …, 2002 - Wiley Online Library
Proteolytic fragments of huntingtin (htt) in human lymphoblast cell lines from HD and control
cases were compared to those in human HD striatal and cortical brain regions, by western …

Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease

CE Wang, S Tydlacka, AL Orr, SH Yang… - Human molecular …, 2008 - academic.oup.com
A number of mouse models expressing mutant huntingtin (htt) with an expanded
polyglutamine (polyQ) domain are useful for studying the pathogenesis of Huntington's …

Elucidating a normal function of huntingtin by functional and microarray analysis of huntingtin-null mouse embryonic fibroblasts

H Zhang, S Das, QZ Li, I Dragatsis, J Repa, S Zeitlin… - BMC neuroscience, 2008 - Springer
Background The polyglutamine expansion in huntingtin (Htt) protein is a cause of
Huntington's disease (HD). Htt is an essential gene as deletion of the mouse Htt gene …

Unconventional secretion and intercellular transfer of mutant huntingtin

BL Tang - Cells, 2018 - mdpi.com
The mechanism of intercellular transmission of pathological agents in neurodegenerative
diseases has received much recent attention. Huntington's disease (HD) is caused by a …