Results 61 to 70 of about 19,163 (188)
Movement Disorders, EarlyView.Abstract Background Although not confirmed, some studies have suggested that elevated homocysteine levels are common in patients with Huntington's disease (HD). Its clinical relevance remains unclear. Objectives We aimed to assess vitamin B and homocysteine levels in HD patients and explore the relationships among hyperhomocysteinemia, vitamin B ...
Salomé Puisieux +16 more
wiley +1 more source
Molecular mechanisms of heterogeneous oligomerization of huntingtin proteins [PDF]
Scientific Reports, 2019 AbstractThere is still no successful strategy to treat Huntington’s disease, an inherited autosomal disorder associated with the aggregation of mutated forms of the huntingtin protein containing polyglutamine tracts with more than 36 repeats. Recent experimental evidence is challenging the conventional view of the disease by revealing transcellular ...
Silvia Bonfanti +7 more
openaire +3 more sources
Proteostasis of organelles in aging and disease
The FEBS Journal, EarlyView.Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi +5 more
wiley +1 more source
Angewandte Chemie, Volume 138, Issue 33, 10 August 2026.In this review, we discuss how biomolecular condensates can inhibit amyloid aggregation in their interior, while still facilitating fibril formation at the interface between the dense and dilute phases, where molecular and mesoscale properties are likely optimal to promote protein aggregation.
Marcell Papp +3 more
wiley +2 more sources
The FEBS Journal, EarlyView.We investigated the potential of iloperidone as an activator of Sigma‐1 receptor (S1R) neuroprotective function in juvenile Huntington's disease (jHD). We tested iloperidone on cortical neurons differentiated from patient‐derived iPSCs, demonstrating that it acts as a S1R agonist, decreasing apoptosis, huntingtin aggregation, and oxidative stress ...
Ersilia Fornetti +11 more
wiley +1 more source
Maintenance of basal levels of autophagy in Huntington's disease mouse models displaying metabolic dysfunction. [PDF]
PLoS ONE, 2013 Huntington's disease (HD) is a fatal neurodegenerative disorder caused by an expanded polyglutamine repeat in the huntingtin protein. Neuropathology in the basal ganglia and in the cerebral cortex has been linked to the motor and cognitive symptoms ...
Barbara Baldo, Rana Soylu, Asa Petersén
doaj +1 more source
The sorting nexin Snx21 promotes flotillin‐mediated endocytosis
The FEBS Journal, EarlyView.In this study, we describe a novel function of the previously uncharacterized Snx21 protein as a specific positive regulator of flotillin‐mediated endocytic trafficking. Snx21 is recruited to endosomal membranes via binding to PI(3)P, and it colocalizes with flotillins on the surface of late endosomes.
Tamás Maruzs +9 more
wiley +1 more source
The Cellular and Subcellular Localization of Huntingtin-Associated Protein 1 (HAP1): Comparison with Huntingtin in Rat and Human [PDF]
The Journal of Neuroscience, 1998 The cellular and subcellular distribution of HAP1 was examined in rat brain by light and electron microscopic immunocytochemistry and subcellular fractionation. HAP1 localization was also determined in human postmortem tissue from control and Huntington’s disease (HD) cases by light microscopic immunocytochemistry.
C A, Gutekunst +5 more
openaire +2 more sources
Stimulating proteasomal degradation in human proteinopathies
The FEBS Journal, EarlyView.The proteasome mediates the degradation of a wide range of proteins. Boosting proteasomal degradation may be beneficial in several disease contexts and can be achieved either by modulating proteasome activity or by improving substrate delivery. Proteasome activity can be enhanced by increasing proteasome abundance, inducing constitutive gate opening ...
Maria E. Gierisch +2 more
wiley +1 more source
20th Anniversary of human‐induced pluripotent stem cells and the role of microscopy
Journal of Microscopy, EarlyView.Abstract It has been 20 years since the pioneering work of Shinya Yamanaka and Kazutoshi Takahashi at Kyoto University led to the first successful generation of induced pluripotent stem cells (iPSCs) from mouse embryonic and adult fibroblast cells. iPSCs have the capacity to differentiate into any type of cell in the human body, and as such, they have ...
Philomena Hallford +3 more
wiley +1 more source