The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington’s disease [PDF]
BMC Research Notes, 2020 Objective Compromised brain cholesterol turnover and altered regulation of brain cholesterol metabolism have been allied with some neurodegenerative diseases, including Huntington’s disease (HD). Following our previous studies in HD, in this study we aim
Clévio Nóbrega +12 more
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Effects of Complete and Partial Loss of the 24S-Hydroxycholesterol-Generating Enzyme Cyp46a1 on Behavior and Hippocampal Transcription in Mouse [PDF]
BiomoleculesBrain cholesterol metabolic products include neurosteroids and oxysterols, which play important roles in cellular physiology. In neurons, the cholesterol oxidation product, 24S-hydroxycholesterol (24S-HC), is a regulator of signaling and transcription ...
Hong-Jin Shu +8 more
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Efavirenz treatment improves retinal vaso-obliteration and pathological neovascularization in a mouse model of retinopathy of prematurity [PDF]
Frontiers in MedicineObjectivesPrevious studies have shown the metabolic and regulatory significance of CYP46A1 in the adult retina; however, its role in the developing retina is unknown. Here, we evaluate CYP46A1 expression and the impact of its activation in the developing
Briah Bailey +9 more
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Astrocyte-neuron combined targeting for CYP46A1 gene therapy in Huntington’s disease [PDF]
Acta Neuropathologica CommunicationsHuntington’s disease (HD) is an autosomal dominant neurodegenerative disease caused by an abnormal expansion of cytosine-adenine-guanosine (CAG) trinucleotidein the huntingtin gene.
Louis-Habib Parsai +15 more
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Structure–Activity Relationship Study on Soticlestat Derivatives for the Discovery of CYP46A1 (CH24H) Inhibitors [PDF]
MoleculesDravet syndrome (DS) and Lennox–Gastaut syndrome (LGS) are rare, severe developmental and epileptic encephalopathies with poor prognosis, and novel drugs are urgently needed to meet clinical needs.
Xinwei Hu +6 more
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Frontiers in Aging Neuroscience, 2020 Polymorphism of the cholesterol-24S-hydroxylase (CYP46A1) gene is thought to be a risk factor for Alzheimer’s disease (AD). A single nucleotide polymorphism (T/C) in intron 2, rs754203, has been confirmed to be implicated in AD.
Fan Zeng, Hua-Dong Zhou, Y-J Wang
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The cholesterol 24-hydroxylase CYP46A1 promotes α-synuclein pathology in Parkinson's disease. [PDF]
PLoS BiologyParkinson's disease (PD) is a neurodegenerative disease characterized by the death of dopaminergic neurons in the substantia nigra and the formation of Lewy bodies that are composed of aggregated α-synuclein (α-Syn).
Lijun Dai +11 more
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Frontiers in Molecular Neuroscience, 2020 The manipulation of cholesterol and its metabolites has been hypothesized to be therapeutically beneficial for mood disorders, neurodegenerative disorders, and epilepsies.
Yukitoshi Izumi +2 more
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Therapeutic implications of altered cholesterol homeostasis mediated by loss of CYP46A1 in human glioblastoma [PDF]
EMBO Molecular Medicine, 2019 Dysregulated cholesterol metabolism is a hallmark of many cancers, including glioblastoma (GBM), but its role in disease progression is not well understood. Here, we identified cholesterol 24‐hydroxylase (CYP46A1), a brain‐specific enzyme responsible for
Mingzhi Han +14 more
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Treatment with efavirenz extends survival in a Creutzfeldt-Jakob disease model by regulating brain cholesterol metabolism [PDF]
JCI InsightPrion diseases are fatal, infectious, and incurable neurodegenerative conditions affecting humans and animals, caused by the misfolding of the cellular prion protein (PrPC) into its pathogenic isoform, PrPSc. In humans, sporadic Creutzfeldt-Jakob disease
Tahir Ali +5 more
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