In Vivo Mapping of Catecholaminergic Loss and Iron Deposition in Huntington's Disease
Abstract Background The pathophysiology of Huntington's disease (HD) remains obscure. Magnetic resonance imaging (MRI) can reveal in vivo molecular changes related to disease pathology. Objectives To investigate catecholaminergic neuronal integrity and subcortical brain iron accumulation in HD employing neuromelanin‐sensitive MRI, and quantitative ...
Edoardo R. de Natale +11 more
wiley +1 more source
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome [PDF]
Expansion of the polyglutamine repeat within the protein Huntingtin (Htt) causes Huntington's disease, a neurodegenerative disease associated with aging and the accumulation of mutant Htt in diseased neurons.
Aiken +97 more
core +3 more sources
The psychosocial impacts of Huntington's disease include disintegration with society, emotional and psychological burdens, an interplay of extrinsic stressors and recalibration of the family system. This thematic synthesis highlights the need for holistic, systemic support to address these pervasive challenges for all members of the affected family ...
Paige Lindo +4 more
wiley +1 more source
The differential diagnosis of Huntington's disease-like syndromes: 'red flags' for the clinician [PDF]
A growing number of progressive heredodegenerative conditions mimic the presentation of Huntington's disease (HD). Differentiating among these HD-like syndromes is necessary when a patient with a combination of movement disorders, cognitive decline ...
Bhatia, KP, Martino, D, Stamelou, M
core
Epidemiology of progressive intellectual and neurological deterioration in UK children
This study of PIND in UK children was carried out via the British Paediatric Surveillance Unit from 1997 to 2024. It identified six cases of vCJD. 2367 children had other diagnoses explain their deterioration. There were 259 other diseases in the diagnosed group.
Christopher M. Verity +3 more
wiley +1 more source
Molecular diagnosis of Huntington disease in Portugal : implications for genetic counselling and clinical practice [PDF]
Huntington disease (HD) is a eurodegenerative, autosomal dominant disorder of late-onset, caused by the expansion of a CAG repeat in the coding region of the gene. Ours is the reference laboratory for genetic testing in HD, in Portugal, since 1998; 90.1%
A Durr +39 more
core +1 more source
Personalized Models of Biological Barriers and Their Diseases: Recent Progress with Organs‐On‐Chips
Buck and Bugter et al. explore the architectural diversity and physiological functions of human barrier systems and reveal how organ‐on‐chip platforms, particularly those integrating patient‐derived cells, are advancing barrier disease modeling. They highlight how emerging biological and technological advances can be used to bridge the gap between ...
Franziska Buck +4 more
wiley +1 more source
Sphingomyelin and GM1 Influence Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes [PDF]
Huntington disease (HD) is an inherited neurodegenerative disease caused by the expansion beyond a critical threshold of a polyglutamine (polyQ) tract near the N-terminus of the huntingtin (htt) protein. Expanded polyQ promotes the formation of a variety
Campbell, Warren A. +5 more
core +4 more sources
Ginsenoside Rg1 (Rg1) from Panax ginseng shows multifunctional health effects. Although oral bioavailability is low and blood–brain barrier (BBB) penetration is limited, delivery systems such as liposomes and nanoparticles may improve exposure. Rg1 reduces inflammation/oxidative stress by inhibiting nuclear factor‐κB (NF‐κB) signaling and reactive ...
Hernán Cortés +13 more
wiley +1 more source
Juvenile‐onset Huntington's disease – Spectrum and evolution of presenting movement disorders
Juvenile‐onset Huntington's disease (HD) is a rare subset of HD with symptom‐onset before the age of 18. In contrast to the adult population, children present early‐on with behavioral, psychiatric, and cognitive symptoms, in addition to a diverse ...
Kathryn Yang +6 more
doaj +1 more source

