Results 61 to 70 of about 4,891,805 (263)

Decoding neuronal vulnerability: Multidimensional analysis of D1R- and D2R- medium-sized spiny neurons in Huntington's disease. [PDF]

Brain Pathol
D1R and D2R MSNs show distinct responses to Huntington's pathology. G&T‐sequencing of pre‐symptomatic MSNs reveals global LINE‐1 downregulation. While D1R‐MSNs display early transcriptomic changes (OXPHOS/translation upregulation) and reduced mutant huntingtin aggregation, D2R‐MSNs exhibit higher nuclear mHTT accumulation and greater CAG somatic ...
Bergonzoni G, Pellegrini M, Savino A, Lazioli M, Geurs S, Graziani L, Ferrarini D, Tusi SK, Oliver E, Geurts J, Vinciguerra S, Tebaldi M, Tripathi T, Pesce I, Poli V, Romanel A, Moratalla R, Voet T, Morandell J, Sanges R, Wheeler VC, Dassi E, Biagioli M.   +22 more
europepmc   +2 more sources

Targeting the cholinergic system to develop a novel therapy for Huntington's disease

, 2016
In this review, we outline the role of the cholinergic system in Huntington's disease, and briefly describe the dysfunction of cholinergic transmission, cholinergic neurons, cholinergic receptors and cholinergic survival factors observed in post-mortem ...
D'Souza, GX, Waldvogel, Henry
core   +1 more source

Huntingtin Aggregate‐Responsive Autophagy Gene Circuit Mitigates Disease Pathology in R6/2 Mice

Advanced Science, EarlyView.
CD98‐mediated receptor‐mediated transcytosis enables LIP‐CD98 nanocarriers to cross the blood–brain barrier and deliver ARAA to neurons. mHTT aggregates activate the 11G–NarX sensor, initiating Auto‐P and Trans‐P signaling through the VP48–NarL relay.
Jie Zhu, Xi‐xiu Xie, Lei Li, Chen Tian, Hao‐tian Wang, Xiao‐jie Wang, Xiao‐lin Yu, Gui‐feng Zhang, Rui‐tian Liu   +8 more
wiley   +1 more source

Neurotransmitter‐Defined Degeneration Patterns in Sporadic and C9orf72‐Associated Amyotrophic Lateral Sclerosis: Predilection to GABAergic, Serotonergic, Opioid, Glutamatergic, Endocannabinoid, and Microglial Systems—Implications for Therapy Development

Annals of Neurology, EarlyView.
Objective Amyotrophic lateral sclerosis (ALS) has a markedly distinctive clinical and neuroradiological signature, with the preferential involvement of specific brain networks and the apparent sparing of others. The molecular underpinnings of the strikingly selective anatomical vulnerability have not been fully elucidated to date despite the potential ...
Marlene Tahedl, We Fong Siah, Jasmin Lope, Jennifer C. Hengeveld, Mark A. Doherty, Russell L. McLaughlin, Orla Hardiman, Ee Ling Tan, Alison Toomey, Jana Kleinerova, Peter Bede   +10 more
wiley   +1 more source

A Case Report: Psychotic Disorder Related to Huntington‘s Disease [PDF]

Düşünen Adam Psikiyatri ve Nörolojik Bilimler Dergisi, 2004
Basal ganglia disorders are characterised by the presence of abnormal movements, psychiatric signs and symptoms, and varying degrees of cognitive impairment. Huntington's disease (HD), one of basal ganglia disorders is is an autosomal, dominant disorder.
Çağatay Karşıdağ, Rıdvan Üney, Ahmet Kocabıyık, Reşit Kükürt, Nihat Alpay, Cengiz Dayan   +5 more
doaj  

Juvenile Huntington’s disease

Анналы клинической и экспериментальной неврологии, 2017
Juvenile Huntingtons disease (JHD) manifests in 1st2nddecades of life and accounts for 29% of all cases ofHuntingtons disease; its pathogenic mechanisms are related togenetic anticipation and imprinting.
G. E. Rudenskaya, D. A. Savvin, V. P. Fedotov, S. A. Kurbatov, A. V. Polyakov, N. M. Galeeva   +5 more
doaj   +1 more source

Multimodal Characterization of Glymphatic‐Related Magnetic Resonance Imaging Markers in Huntington's Disease: A Multi‐Cohort Retrospective Study

Annals of Neurology, EarlyView.
Objective To characterize magnetic resonance imaging (MRI)‐based glymphatic surrogates in Huntington's disease (HD) using MRI measures of perivascular diffusivity and structural perivascular alterations across multiple large cohorts. Methods We analyzed 2,731 MRI sessions from 880 participants across 3 large retrospective HD cohorts.
Alexia Solomon, Zexi Wang, Francesco Sanvito, Zheng Zhou, Adys Mendizabal, Jingwen Yao   +5 more
wiley   +1 more source

Precise CAG repeat contraction in a Huntington’s Disease mouse model is enabled by gene editing with SpCas9-NG

Communications Biology, 2021
Seiya Oura and Taichi Noda et al. overcome the challenge of gene editing in CAG repeats, such as those causing Huntington’s Disease, using their recently developed SpCas9-NG variant. They demonstrate that SpCas9-NG can precisely edit and contract the CAG
Seiya Oura, Taichi Noda, Naoko Morimura, Seiji Hitoshi, Hiroshi Nishimasu, Yoshitaka Nagai, Osamu Nureki, Masahito Ikawa   +7 more
doaj   +1 more source

Availability and Pricing of Orthobiologic Knee Injections Vary by Practice Type but Not by Community Socioeconomic Vulnerability in Suburban New York

Arthroscopy, Sports Medicine, and Rehabilitation, EarlyView.
Purpose To assess the availability, price transparency, and pricing of platelet‐rich plasma (PRP), bone marrow aspirate concentration, and stem cell injections for knee osteoarthritis (OA) among orthopaedic sports medicine (OSM) practices and alternative clinics in suburban New York and to evaluate the impact of the economic status of a population on ...
Tracy M. Tauro, Benjamin Hershfeld, Katharine Chen, Ran Lampert, Adam D. Bitterman, Randy M. Cohn, Nicholas A. Sgaglione   +6 more
wiley   +1 more source

A 24-Hour Study of the Hypothalamo-Pituitary Axes in Huntington's Disease. [PDF]

PLoS ONE, 2015
Huntington's disease is an inherited neurodegenerative disorder characterised by motor, cognitive and psychiatric disturbances. Patients exhibit other symptoms including sleep and mood disturbances, muscle atrophy and weight loss which may be linked to ...
Eirini Kalliolia, Edina Silajdžić, Rajasree Nambron, Seán J Costelloe, Nicholas G Martin, Nathan R Hill, Chris Frost, Hilary C Watt, Peter Hindmarsh, Maria Björkqvist, Thomas T Warner   +10 more
doaj   +1 more source