Niemann-Pick disease type C symptomatology: an expert-based clinical description
E Mengel, HH Klünemann, CM Lourenço… - Orphanet journal of rare …, 2013 - Springer
Niemann-Pick disease type C (NP-C) is a rare, progressive, irreversible disease leading to
disabling neurological manifestations and premature death. The estimated disease …
disabling neurological manifestations and premature death. The estimated disease …
[HTML][HTML] Direct and indirect cholesterol effects on membrane proteins with special focus on potassium channels
As described in the literature the interaction between cholesterol and membrane proteins
can occur via direct, ligand-like and indirect mechanisms, in which cholesterol effects are …
can occur via direct, ligand-like and indirect mechanisms, in which cholesterol effects are …
Closed-state inactivation and pore-blocker modulation mechanisms of human CaV2. 2
Y Dong, Y Gao, S Xu, Y Wang, Z Yu, Y Li, B Li, T Yuan… - Cell reports, 2021 - cell.com
N-type voltage-gated calcium (Ca V) channels mediate Ca 2+ influx at presynaptic terminals
in response to action potentials and play vital roles in synaptogenesis, release of …
in response to action potentials and play vital roles in synaptogenesis, release of …
Cyclodextrins and iatrogenic hearing loss: new drugs with significant risk
MA Crumling, KA King, RK Duncan - Frontiers in cellular …, 2017 - frontiersin.org
Cyclodextrins are a family of cyclic oligosaccharides with widespread usage in medicine,
industry and basic sciences owing to their ability to solubilize and stabilize guest …
industry and basic sciences owing to their ability to solubilize and stabilize guest …
Molecular insights into the gating mechanisms of voltage-gated calcium channel CaV2.3
Y Gao, S Xu, X Cui, H Xu, Y Qiu, Y Wei, Y Dong… - Nature …, 2023 - nature.com
High-voltage-activated R-type CaV2. 3 channel plays pivotal roles in many physiological
activities and is implicated in epilepsy, convulsions, and other neurodevelopmental …
activities and is implicated in epilepsy, convulsions, and other neurodevelopmental …
Hearing loss and hair cell death in mice given the cholesterol-chelating agent hydroxypropyl-β-cyclodextrin
MA Crumling, L Liu, PV Thomas, J Benson, A Kanicki… - PloS one, 2012 - journals.plos.org
Cyclodextrins are sugar compounds that are increasingly finding medicinal uses due to their
ability to complex with hydrophobic molecules. One cyclodextrin in particular, 2 …
ability to complex with hydrophobic molecules. One cyclodextrin in particular, 2 …
[HTML][HTML] Molecular interaction and functional coupling between type 3 inositol 1, 4, 5-trisphosphate receptor and BKCa channel stimulate breast cancer cell …
A Mound, L Rodat-Despoix, S Bougarn… - European journal of …, 2013 - Elsevier
Background The implication of ion channels and inositol 1, 4, 5-trisphosphate (IP 3)-induced
Ca 2+ signalling (IICS) in the carcinogenesis processes, including deregulation of cell …
Ca 2+ signalling (IICS) in the carcinogenesis processes, including deregulation of cell …
[HTML][HTML] Depletion of membrane cholesterol modifies structure, dynamic and activation of Nav1. 7
S Albani, VSB Eswaran, A Piergentili… - International Journal of …, 2024 - Elsevier
Cholesterol is a major component of plasma membranes and plays a significant role in
actively regulating the functioning of several membrane proteins in humans. In this study, we …
actively regulating the functioning of several membrane proteins in humans. In this study, we …
Effects of cholesterol on nano-mechanical properties of the living cell plasma membrane
In this study, we investigated the effects of membrane cholesterol content on the mechanical
properties of cell membranes by using optical tweezers. We pulled membrane tethers from …
properties of cell membranes by using optical tweezers. We pulled membrane tethers from …
Niemann-Pick type C disease reveals a link between lysosomal cholesterol and PtdIns (4, 5) P2 that regulates neuronal excitability
There is increasing evidence that the lysosome is involved in the pathogenesis of a variety
of neurodegenerative disorders. Thus, mechanisms that link lysosome dysfunction to the …
of neurodegenerative disorders. Thus, mechanisms that link lysosome dysfunction to the …