Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3 [PDF]
Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an autosomal-dominant neurodegenerative disorder caused by a polyglutamine expansion in ataxin-3 (SCA3, MJD1) protein.
Bezprozvanny, I +7 more
core +1 more source
Plasma Glial Fibrillary Acidic Protein Correlates With Brain Metal Burden in Wilson's Disease
ABSTRACT Objective Neuroinflammation driven by extracellular copper contributes to neuronal damage in Wilson's disease (WD). This study investigated the relationship between brain metal burden and peripheral neuroinflammation markers in WD. Methods We conducted a cross‐sectional study involving 89 participants, including patients with WD (n = 63 ...
Sung‐Pin Fan +12 more
wiley +1 more source
Late-onset Parkinsonism in NFκB/c-Rel-deficient mice. [PDF]
Activation of the nuclear factor \u3baB/c-Rel can increase neuronal resilience to pathological noxae by regulating the expression of pro-survival manganese superoxide dismutase (MnSOD, now known as SOD2) and Bcl-xL genes.
Alghisi M +17 more
core +3 more sources
Immunocytochemical localization of glutamate decarboxylase in rat substantia nigra [PDF]
l-Glutamate decarboxylase (GAD, EC 4.1.1.15), the enzyme which catalyzes the α-decarboxylase of l-glutamate to form γ-aminobutyric acid (GABA), was localized both light and electron microscopically in rat substantia nigra by an immunoperoxidase method ...
Barber, R +4 more
core +1 more source
CSF Mitochondrial DNA: Biomarker of Body Composition and Energy Metabolism in Parkinson's Disease
ABSTRACT Objective Cerebrospinal fluid (CSF) cell‐free mitochondrial DNA (cf‐mtDNA) is a potential biomarker for Parkinson's disease (PD), but its clinical relevance remains unclear. We investigated associations between CSF cf‐mtDNA levels, body composition, nutritional status, and metabolic biomarkers in PD. Methods CSF cf‐mtDNA levels, defined as the
Yasuaki Mizutani +11 more
wiley +1 more source
The cell cycle–apoptosis connection revisited in the adult brain [PDF]
Adult neurogenesis is studied in vivo using thymidine analogues such as bromodeoxyuridine (BrdU) to label DNA synthesis during the S phase of the cell cycle.
Bauer, Sylvian, Patterson, Paul H.
core +3 more sources
Neural connectivity predicts spreading of alpha-synuclein pathology in fibril-injected mouse models: Involvement of retrograde and anterograde axonal propagation. [PDF]
In Parkinson's disease, some of the first alpha-synuclein aggregates appear in the olfactory system and the dorsal motor nucleus of the vagus nerve before spreading to connected brain regions.
Brundin, Patrik +3 more
core +2 more sources
Purkinje Cell Loss in Essential Tremor: Collective Data From 215 Brains Over a 21‐Year Period
ABSTRACT Objective Essential tremor is a highly prevalent movement disorder. Pathological changes observed in essential tremor cerebella center around Purkinje cells and neighboring neuronal populations. Postmortem studies have variably, but not always, shown reduced Purkinje cell counts in essential tremor compared to controls.
Chloë A. Kerridge +4 more
wiley +1 more source
A hallmark of Parkinson's disease is the specific degeneration of dopaminergic neurons in the substantia nigra pars compacta. Interestingly, not all of these neurons are affected to the same extent.
Simone Steinbach +8 more
doaj +1 more source
MPTP-induced degeneration: interference with glutamatergic toxicity [PDF]
Parkinson's disease (PD) is characterised by the progressive degeneration of nigrostriatal dopamine (DA) neurons resulting in the major symptoms of akinesia and rigidity.
Lange, Klaus W. +3 more
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