[HTML][HTML] A huntingtin knockin pig model recapitulates features of selective neurodegeneration in Huntington's disease
Huntington's disease (HD) is characterized by preferential loss of the medium spiny neurons
in the striatum. Using CRISPR/Cas9 and somatic nuclear transfer technology, we …
in the striatum. Using CRISPR/Cas9 and somatic nuclear transfer technology, we …
[HTML][HTML] Elevated TREM2 gene dosage reprograms microglia responsivity and ameliorates pathological phenotypes in Alzheimer's disease models
Variants of TREM2 are associated with Alzheimer's disease (AD). To study whether
increasing TREM2 gene dosage could modify the disease pathogenesis, we developed …
increasing TREM2 gene dosage could modify the disease pathogenesis, we developed …
[HTML][HTML] Protein co-aggregation related to amyloids: methods of investigation, diversity, and classification
SA Bondarev, KS Antonets, AV Kajava… - International journal of …, 2018 - mdpi.com
Amyloids are unbranched protein fibrils with a characteristic spatial structure. Although the
amyloids were first described as protein deposits that are associated with the diseases …
amyloids were first described as protein deposits that are associated with the diseases …
Molecular insights into cortico-striatal miscommunications in Huntington's disease
MB Veldman, XW Yang - Current opinion in neurobiology, 2018 - Elsevier
Highlights•Diverse mouse models of HD have important phenotypic and molecular
similarities.•Disconnectivity of the cortico-striatal synapse drives the disease …
similarities.•Disconnectivity of the cortico-striatal synapse drives the disease …
Brain mitochondrial iron accumulates in Huntington's disease, mediates mitochondrial dysfunction, and can be removed pharmacologically
Mitochondrial bioenergetic dysfunction is involved in neurodegeneration in Huntington's
disease (HD). Iron is critical for normal mitochondrial bioenergetics but can also contribute to …
disease (HD). Iron is critical for normal mitochondrial bioenergetics but can also contribute to …
[HTML][HTML] Profilin reduces aggregation and phase separation of huntingtin N-terminal fragments by preferentially binding to soluble monomers and oligomers
Huntingtin N-terminal fragments (Htt-NTFs) with expanded polyglutamine tracts form a range
of neurotoxic aggregates that are associated with Huntington's disease. Here, we show that …
of neurotoxic aggregates that are associated with Huntington's disease. Here, we show that …
[HTML][HTML] Brain regions showing white matter loss in Huntington's disease are enriched for synaptic and metabolic genes
P McColgan, S Gregory, KK Seunarine, A Razi… - Biological …, 2018 - Elsevier
Background The earliest white matter changes in Huntington's disease are seen before
disease onset in the premanifest stage around the striatum, within the corpus callosum, and …
disease onset in the premanifest stage around the striatum, within the corpus callosum, and …
[HTML][HTML] Regulatory mechanisms of incomplete huntingtin mRNA splicing
A Neueder, AA Dumas, AC Benjamin… - Nature communications, 2018 - nature.com
Huntington's disease is caused by a CAG repeat expansion in exon 1 of the HTT gene. We
have previously shown that exon 1 HTT does not always splice to exon 2 producing a small …
have previously shown that exon 1 HTT does not always splice to exon 2 producing a small …
Time-restricted feeding improves circadian dysfunction as well as motor symptoms in the Q175 mouse model of Huntington's disease
Huntington's disease (HD) patients suffer from a progressive neurodegeneration that results
in cognitive, psychiatric, cardiovascular, and motor dysfunction. Disturbances in sleep/wake …
in cognitive, psychiatric, cardiovascular, and motor dysfunction. Disturbances in sleep/wake …
Transcriptional regulatory networks underlying gene expression changes in Huntington's disease
Transcriptional changes occur presymptomatically and throughout Huntington's disease
(HD), motivating the study of transcriptional regulatory networks (TRN s) in HD. We …
(HD), motivating the study of transcriptional regulatory networks (TRN s) in HD. We …